10⼀Heme-Onc/TOX I Flashcards
⬜ are 4 major triggers of vasooclusive crisis in Sickle Cell Disease patients
What is the treatment for vasooclusive crisis? (5)
_________________
Which med is used for long term management?
109DICK (Dehydration / Infection / Cold temp / [Kant breathe (hypoxia)])
_________________
acute tx = Rehydration / [abx and PAIN CONTROL (NSAID>opioids)] / heat / oxygen
_________________
chronic tx = Hydroxyurea
Which drugs cause Agranulocytosis? (6)
Gangs Can Certainly Crush Myeloblast & Promyelocytes
Ganciclovir
Clozapine
Carbamazepine
Colchicine
Methimazole(also Teratogenic–>Cutis Aplasia)
PTU
Victims of [smoke inhalation injury] should empircally be treated for Carbon Monoxide and what other chemical toxicity?
________________
What are the treatments for these toxicities? (4)
CYanide toxicity (➜ SEVERE lactic acidosis)
________________
“CYaMonoxide toxicity Needs Overt Smoke Help”
empiric tx for CYanide and (Carbon)Monoxide toxicity
- [Nitrites (induces methemoglobinemia)]
- Oxygen 100% (CO tx)
- [Sodium thiosulfate]
- HydroxoCobalamin (binds Cyanide ➜ excretable Cyanocobalamin)
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
Pulse Ox measures ⬜ which = ⬜
What does [PulseOx] read during methemoglobinemia?
________________
why?
**POSOSOP**
<sub>**P**ulse **O**x =</sub> **S**<sub>p</sub>**O**<sub>2 = </sub> [**S**aturation of **O**<sub>2</sub> on **P**eripheral<sub>RBC</sub>]
* * *
**{low SpO2 < 85%}** = low **[**PulseOx<sup> </sup>S<sub>p</sub>O<sub>2_</sub>**RBC O2 Saturation**]<sub> </sub>\< 85%**}**
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
⚠️*NOTE*: Even though [PaO<sub>2</sub> *oxygen partial pressure*] may read "normal", methemoglobin has lower affinity to O2 than Hgb = Mgb low binding to O2 ➜ **low [RBC O2 saturation]** = **{low POSOROS** = **[**PulseOx<sup> </sup>S<sub>p</sub>*O<sub>2_</sub>**Saturation*]<sub> </sub>\< 85%**}**
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
*Dark chocolate colored blood*
## Footnote
[🔎SpO2 = Saturation of O2 in periphery/vein (via PulseOx)]
[ 🔎SaO2 = Saturation of O2 in **a**rtery (via ABG) <sub>= more accurate</sub>]
🫁<sub>**PaO2<sub>*ABG*</sub>** specifically refers to pressure solely exerted by dissolved oxygen in the arterial blood. It represents amount of oxygen actually dissolved in the plasma of arterial blood, rather than the amount that is bound to hemoglobin (which is reflected in measures like **SaO2<sub>*ABG*</sub>** or **SpO2<sub>*PulseOx*</sub>**).</sub>
Dark chocolate colored blood = ⬜
________________
MOA for tx? (3)
Methemoglobinemia
________________
1st: [NADPH gives electron to [METHYLENE BLUE] ]
2nd: this converts [METHYLENE BLUE] –> [LeukoMethylene blue]
3rd: [LM reduces Methomoglobin –> back to Hgb]
s/s of [hypOcalcemia < 0.76 iCal] (7)
TQT + BOD + Chvostek
_________________
low calcium tx = IV CaGLUCONATE or CaChloride
SEVERE lead toxicity = [(⬜serum lead level) or ⬜]
tx? (2)
≥70 (or encephalopathy)
Moderate lead toxicity = serum lead level of ⬜.
What’s unique about this level of toxicity ?
tx?
[Moderate lead toxicity = 45-69]
[XR lead lines from lead deposition on long bone metaphysis]
_________________
normal lead level = < 5
mild lead toxicity = serum lead level of ⬜
tx? (2)
5-44
tx = [no meds] and [repeat venous blood lead level in 1 month]
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
*normal lead level = \< 5*
Lead inhibits ⬜ which causes what effect on RBC?
major features of lead toxicity -10
[ferrochelatase and ALAD] ; [⬇︎heme synthesis and ⇪ RBC protoporphyrin]
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
**LL**\_**EE**\_**AA**\_**DD\_SS**
1-{**L**ead lines ([Burton's gingival lines] and on [metaphyses of long bones on xr]}
2-[A**L**AD and ferrochelatase] are inhibited by Lead
3-**E**ncephalopathy
4-**E**rythrocyte Basophilic Stippling (RBC retained rRNA aggregates due to Lead inhibiting rRNA degradation)
5-**A**bd colic
6-sideroblastic **A**nemia
7-**D**rops (wrist drop, foot drop, stocking glove)
8-[**D**imercaprol and EDTA are 1st line tx
9-***S**uc*cimer = peds tx ("it *Suc*ks to be a kid who ate lead")
10-**S**eventy-eight ⼀Houses older than 1978 have ⇪ for lead poisoning
## Footnote
*normal lead level < 5*
Iron deficiency is the most common pediatric nutritional deficiency and should be suspected in any child drinking greater than ⬜ cow’s milk /day
___________________
what is the order of physiological changes that occur after giving [ferrous sulfate] iron therapy?
>710 cc
_________________
[ferrous sulfate oral therapy] ➜ ([⇪ reticulocytes] ➜ [⇪ hct & hgb] ) by 1 mo
etx
Aplastic Crisis
AC = [AplasticCrisis)] = [Anemia in Sickle Cell pts,] 2/2 to parvoB19 replicating in (ProErythroblast_Erythroid precursors) → causes [⬇reticulocyte RBC] → [SEVERE ⬇︎Erythropoiesis = (⬇︎RBC/⬇︎Hgb)] … in setting of already (sickle cell) anemic state = Crisis
{[anemia] from parvoB19} occurring in the presence of another (I.e. sickle cell) anemia = “Crisis” because RBC reserve will already be reduced from the other (sickle cell) anemia
What’s the difference between [Aplastic Crisis] and [Aplastic Anemia]?
AA = [(Aplastic Anemia)] =[All-cell PANcytopenia (including ⬇︎Erythropoiesis)] from [failure/destruction/suppression] of [Hematopoietic CD34 Myeloid Stem Cells] By [Bone Marrow T-cells( → hypOcellular bone marrow with fatty infiltration) = dry bone marrow tap] … These [Bone Marrow T-cells] are activated by [IDIOPATHIC > Myelotoxic Drugs/Body Radiation/Virus/Fanconi)]
_________________
AC = [AplasticCrisis)] = [Anemia in Sickle Cell pts,] 2/2 to parvoB19 replicating in (ProErythroblast_Erythroid precursors) → causes [⬇reticulocyte RBC] → [SEVERE ⬇︎Erythropoiesis (⬇︎RBC/⬇︎Hgb)] … in setting of already (sickle cell) anemic state = Crisis
[A C] from parvoB19 is worst in the presence of other anemias since RBC reserve will be reduced
cp for Radiation proctitis (4)
- s/p pelvic radiation therapy
- tenesmus
- bloody diarrhea
- [anal mucus discharge]
ITP
tx for Adults? (4)
[Immune Thrombocytopenic Purpura (ITP)
-obs(cutaneous sx but platelet≥30k )
-[CTS | IVIG | antiD](if bleeding | platelet< 30k)
etx: Ab binds to platelet → both removed by Spleen macrophages → thrombocytopenia → purpura from uncontrolled bleeding
ITP
tx for peds? (4)
[Immune Thrombocytopenic Purpura (ITP)
-obs(if cutaneous sx only )
-[CTS | IVIG | antiD](if bleeding)
etx: Ab binds to platelet → both removed by Spleen macrophages → thrombocytopenia → purpura from uncontrolled bleeding
MethanOL overdose antidote
Fomepizole
_________________
inhibits [hepatic alcohol dehydrogenase] from converting EG/methanol ➜ nephrotoxic metabolites
[Ethylene Glycol (antifreeze)] overdose antidote
Fomepizole
_________________
inhibits [hepatic alcohol dehydrogenase] from converting EG/methanol ➜ nephrotoxic metabolites
In a hemorrhaging patient, when do you transfuse [pRBC]? (2)
_________________
pRBC = packed RBC
hgb< 7
(or < 8 if CVD/CA)
What are the early sx of Sickle Cell disease ? (2)
_________________
how is this diagnosed?
-dactylitis (painful swelling of hands/feet 2/2 bone infarction)
-hemolytic anemia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
<sub>Electrophoresis</sub>: {[Hgb **S**] with [NO Hgb **A**]}
Name the lab pair used to confrim SLE diagnosis
dana Smith
antidsDNA + anti-Smith
Hydoxychloroquine is a ⬜ used to treat what sx in SLE? (3)
_________________
How is CTS used in SLE patients? (2)
anti-malarial ;
Rash / Arthralgia / Soft tissue synovitis
_________________
acute SLE = low dose CTS
Chronic/SEVERE SLE = HIGH dose CTS
RA is a common cause of Anemia of Chronic Disease
How do you treat ACD 2/2 RA?
Infliximab(AntiTNFα)
{ACD🔧: chronic/inflammation→ hepcidin→ [macrophage/intestinalferroportin]❌ from transfering Fe from FerriTin to Transferrin ➜ {[ ↧ Transferrin Saturation with paradoxic ⬇︎TIBC] , [↥ FerriTin],} and [⬇︎Fe**in circulation*],
Upregulators of Cytochrome P450 Enzymes (8)
Chronic alcoholics Steal Phen-Phen & Never Refuse Greasy Carbs which keeps them UP
- Chronic alcohol use
- St.John’s wort
- Phenytoin
- Phenobarbital
- Nevirapine
- Rifampin
- Griseofulvin
- Carbamazepine
Inhibitors of Cytochrome P450 Enzymes (11)
AAA RACKS In GQ Magazine
INHIBIT me from doing my job!
Acute Alcohol Abuse
Ritonavir (HIV Protease inhibitor)
Amiodarone
[Cimetidine & Ciprofloxacin]
Ketoconazole
Sulfonamides
[INH Isoniazid]
[Grapefruit Juice]
Quinidine
[Macrolides (except Azithro)]
What Substrates does Cytochrome P450 metabolize? (6)
Can Always Think When Outdoors, Son….i need it!
[Cyclosporine (Liver AND small intestine)]
AntiEpileptics
Theophylline
Warfarin
OCP
[Statins (NOT PRAVASTATIN)]
M.D. must recognize early signs of [Phenytoin toxicity > ⬜ mcg/ml]
What’s the earliest sign?
20
[Nystagmus on far lateral gaze]
_________________
other signs: [diplopia, ataxia ➜ coma]
[Phenytoin 10-(therapeutic)-20 < (TOXIC)]
Tx for Clostridium Botulinum poisoning - 3
- [Equine Heptavalent Anti- Botulinum toxin (passive immunity)]
- [Ig Anti- Botulinum]
- Guanidine
Jimson Weed Poisoning clinical presentation - 7
Jimson Weed = AntiCholinergic
“Blind as a bat, mad as a hatter, red as a beet, hot as a hare, dry as a bone, the bowel & bladder lose their tone, and the heart runs alone…..”
- Blind as a bat = [Mydriasis and [cycloplegia (blurry vision especially when focusing on near objects)]
- Mad as a hatter= Agitation & Hallucinations
- Red as a Beet = Cutaneous flushing despite vasoconstriction
- Hot as a hare = Hyperthermia from DEC ability to sweat
- Dry as a bone= DEC Secretions (including sweat)
- Bladder & Bowel lose tone
- Heart runs alone = No vagal tone at SA –> Tachycardia
[MMalignant Hyperthermia] etx
After giving [inhaled anesthestics vs succinylcholine] to genetically predisposed (AUTO DOM) pts develop ➜
Malignant = Muscle Rigidity
Malignant = Malignant Unstable Vitals
Hyperthermia = Fever
MMalignant Hyperthermia Tx
Dantrolene
TREAT PROMPTLY! AS THIS IS LIFE THREATENING CONDITION!
TTP treatment
_________________
TTP = Thrombotic Thrombocytopenic Purpura
plasma EXCHANGE
_________________
(Tx = EXCHANGE plasma containing anti-A13vM or deficiency of A13vM for normal plasma)
A13vM= [ADAMTS-13-vWF Metalloprotease])
TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → FMNRT TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ (TTP Tx = EXCHANGE plasma containing anti-A13vM or deficiency of A13vM for normal plasma)
TTP MOD (4)
_________________
TTP = Thrombotic Thrombocytopenic Purpura
① {[A13vM (ADAMTS13_vWF Metalloprotease)] (responsible for degrading vWF Multimers)}
② A13vM❌ = [acquired A13vM inhibition(by shiga toxin in HUS)] or [ acquired A13vM deficiency] (2/2 CLOpidogrel | tiCLOpidine | cyCLOsporine | AIDS )
③ ➜ allows ⇪ vWF Multimers to circulate and start cleaving large chains of von Willebrand factor from the vascular endothelium ➜ cleaved vWF ➜ widespread platelet traps and platelet activation ➜severe thrombocytopenia
④ +{RBC shearing ➜ [Schistocyte helmet cells], [INC Direct bilirubin], [microangiopathic hemolytic anemia]}
☞ TTP Symptom PENTAD (FMNRT)
_________________
tx = plasma EXCHANGE {(exchanges [anti-A13vM] and/or [low A13vM])for [more A13vM])}
💡in HUS, shiga toxin inhibits A13vM → similar TTP (but w/out neuro sx or plasma EXCHANGE tx) clinical presentation
In RBC…
Adult Hemoglobin is made of 4 globin chains. What are they? fetal Hemoglobin?
_________________
βeta Thalassemia occurs because of ⬜ on Chromosome ⬜ of either parent. This → β-chain being ⬜
_________________
describe the 2 variants of βeta thalassemia
βeta Thalassemia = SEVERE anemia
hgbA_ADULT[αα⼀αα / β⼀β] hgbF_fetal[αα⼀αα / γ⼀γ]
[point mutations in splice & promoter sequences]; 11; [underproduced or ABSENT]
[αα⼀αα / ❌⼀β] = βTminor (➜ β-chain underproduced= asx, [⇪ HgbA2= dx], )
_________________
[αα⼀αα / ❌⼀❌] = βTMAJOR (➜ β-chain ABSENT = early death , ⇪ HgbF|| temp tx = Hypertransfusion)
_________________
- *αα (2 genes) = 1 α globin chain* | βT = βeta Thalassemia*
- causes microcytic hypOchromic anemia*
In RBC…
[Adult Hemoglobin A] is made of 4 globin chains. What are they? fetal Hemoglobin?
_________________
αlpha thalassemia occurs due to ⬜ on chromosome ⬜. This can ultimately → 5 variants of hemoglobin makeup
Describe all 5 αT variants
alpha Thalassemiaa
hgbA_ADULT[αα⼀αα / β⼀β] hgbF_fetal[αα⼀αα / γ⼀γ]
[αlpha allele deletion (2 alleles per parent);] chromo 16
_________________
I:[⬜α⼀αα / β⼀β] ➜ [αT⼀silent carrier]
II:[⬜α⼀⬜α / β⼀β] ➜ [αT⼀traitTRANS]
II:[⬜⬜⼀αα / β⼀β] ➜ [αT⼀traitCIS(worst for offspring)]
III:[⬜⬜⼀⬜α / β⼀β] ➜ [αT⼀hb]
IV:[⬜⬜⼀⬜⬜/ β⼀β] ➜ [αT⼀🅱🅶]
_________________
_________________
[αT⼀hb] = Hb(h)**tetramer of beta]
[αT⼀🅱🅶]= {[h🅱:T🅶 :HFD]} = {Hgb** 🅱ART tetramer of 🅶amma (Hydrops Fetalis(DIES IN UTERO))**]}
*αα (2 genes) = 1 α globin chain* | αT = alpha Thalassemia
[Microcytic hypOchromic anemia] likely indicates the congenital hemolytic anemia ⬜.
Why are these patients at risk for organ damage 2/2 iron overload ? (3)
[Beta Thalassemia MAJOR [αα⼀αα / ❌⼀❌]] ;
Hypertransfusion tx overcomes effects of anemia and extramedullary hematopoiesis BUT also ➜ iron overload ➜ severe organ damage from iron deposition
[Anemia with Normal RDW] typically indicates ____
Thalassemia
What is Cooley Anemia?
________________
tx?
Beta thalassemia MAJOR (BOTH Mom and Dad [chromo 11 β-globin genes] have [splice/promoter point mutations] →2 out of 2 ABSENT β-globin → [⇪ HgbF (a2g2)] = SEVERE COOLEY ANEMIA
________________
Tx = [chronic blood transfusion with deferadirox iron chelator]
DDx - 4
Target cells = HALT
- HbC
- Asplenia
- Liver disease
- Thalassemia (usually asx and REQUIRES NO TX if asx - occurs in Mediterranean people)
Dx
Thalassemia
image shows teardrop cells (Thalassemia also has Target cells)
What must you be VERY CAUTIOUS of when treating Megaloblastic macrocytic anemia with Vitamin B12?
Explain
hypOkalemia! (within 48h of VB12 tx)
_________________
VB12 supplement in moderate/Severe Megaloblastic macrocytic anemia will ➜ newly formed RBC ➜ RAPID INTRACELL UPTAKE OF K+ by new RBC ➜ hypOkalemia!
So…
[transfuse pRBC before VB12 ➜ blunts new RBC synthesis ➜ prevents hypOkalemia] + [monitor K+ 48h]
clinical presentation of neonatal polycythemia (5)
- neonatal [PERIPHERAL venous hct > 65%]
- **[ASX v 24h self limited] **
- [+/- life threatening apnea]
- [+/- hypOglycemia]
- [+/- hyperviscosity (causes hypOperfusion ➜ lethargy, hypOtonia)]
_________________
1st hct comes from heel prick but = unreliable / confirmed by peripheral venous if abnml
treatment for neonatal polycythemia (3)
- [HYDRATION]
2. CORRECT METABOLIC DERANGEMENT
- –(if persist)–> [Partial exchange transfusion]
Patients with Multiple Myeloma need skeletal system assay for bone involvement
How is this done?
[Whole-Body-Cross-Sectional] eval with
[low-dose CT no contrast] > MRI or PET
What is hyperviscosity syndrome?
excessive production of monoclonal IgM (Waldenstrom Macroglobulinemia > Multiple Myeloma) ➜ congestion of brain’s microcirculation➜ [neuro ∆ /hearing ∆ /vision ∆]
_________________
neuro ∆ = somnolence/coma/HA
tx = plasmapheresis
There are several causes of Transiently elevated PSA, in which PSA should DEC by ⬜
What are the 3 causes of Persistently elevated PSA?
4-6 wks;
- [CPCPPS]
- BPH
- Prostate CA
___________________________x____________________________________
(Chronic Prostatitis⼀Chronic Pelvic Pain Syndrome)
What do patients use Ginkgo biloba for?
_________________
What’s the major potential side effect?
[“memory booster” (2/2 to its suggested propensity for ⇪ cerebral blood flow)]
_________________
Bleeding(inhibits platelet-activating factor + potentiates anticoagulants)
(especially if combined with ASA/antiplatelet drugs)
“[herbs GSG] cause Bleeds”
Explain the relationship between [Tumor Lysis Syndrome] and Cardiac arrest (6)
Chemotherapy commonly ➜ {TLS = [⇪PUKED (and ⬇︎Ca+)]} –(if SEVERE hyperKalemia)–> [sine wave Wide QRS] ➜ VENTRICULAR ASYSTOLE ➜ CARDIAC ARREST
PUK = Phosphate/Uric acid/K+
FFP is used for what purpose? (2)
- DIC
- 2nd line tx for[Vitamin K deficiency coagulopathy(i.e. warfarin OD INR ≥2)] ;
(2nd line tx for Warfarin OD because FFP requires large volume > 2L)
When is Platelet transfusion indicated?
[platelet < 50K]
Laboratory findings for [Cobalamin VB12] deficiency (3)
- PANcytopenia
- Macrocytic anemia
- low reticulocyte count
common cause of delirium/dementia in elderly!
Vegan Elderly patients p/w delirium or dementia should always make you c/f ⬜
[Cobalamin B12] deficiency!
Describe the 2 molecular reactions [Cobalamin B12] is responsible for
How does [Cobalamin VitB12] deficiency lead to hyperbilirubinemia
[Cobalamin VitB12 deficiency] ➜ defective DNA synthesis in bone marrow ➜ [Erythroid hyperplasia but with no maturation] = RBC megaloblastic transformation ➜ [intramedullary RBC hemolysis] ➜ [⇪ hemolysis markers ⼀indirect bilirubinemia, LDH, (low haptoglobin)] and no reticulocyte response
how do you emergently reverse Warfarin OD? (2)
[PCC +KIV] > ffp
_________________
- PCC = Prothrombin Concentrate Complex (contain VitK factors and normalizes INR<10m) / KIV = Vitamin K IV(takes 12h to onset)*
- ffp= fresh frozen plasma(2nd line due to large volume >2L required)*
These 3 agents emergently reverse ⬜
Briefly Describe each
a. [PCC ⼀ProThrombin Complex Concentrate]
b. [KIV ⼀Vitamin K IV]
c. [FFP ⼀Fresh Frozen Plasma]
WARFARIN
[PCC +KIV] > ffp
_________________
a. PCC = contain [VitK factors2/7/9/10] and normalizes INR<10m
b. KIV = takes 12h to onset
c. ffp = 2nd line for warfarin reversal due to [large volume >2L required]
Warfarin
MOA
Treatment for Warfarin toxicity depends on INR and Bleeding
Name the [Warfarin toxicity] tx* (2) *for:
[(supratherapeutic) INR: 3-4.5]
with
[Bleeding: ≤minimal]
[Hold Warfarin x 1-2d] | [DEC dose]
Treatment for Warfarin toxicity depends on Bleeding and INR
Name the [Warfarin toxicity] tx* (2) *for:
[Bleeding: ≤minimal]
with
[(supratherapeutic) INR: 4.5-10]
- Hold Warfarin ➜ Resume when INR therapeutic
- [vitKLd 1- 2.5mg PO](If INC risk of bleeding)**
_________________
Treatment for Warfarin toxicity depends on INR and Bleeding
Name the [Warfarin toxicity] tx* (2) *for:
[(supratherapeutic) INR: >10]
with
[Bleeding: ≤minimal]
- Hold Warfarin ➜ Resume when INR therapeutic
- GIVE [vitKHD 2.5 - 5 mg PO]
_________________
Treatment for Warfarin toxicity depends on INR and Bleeding
Name the [Warfarin toxicity] tx* (3) *for:
[INR: x]
with
[Bleeding: SEVERE]
- Hold Warfarin ➜ Resume when INR therapeutic
- GIVE [vitKUHD10 mg IV]
- GIVE [PCC]
_________________
What is Pernicious Anemia?
_________________
how is this diagnosed?
[Anti-ParietalCell and Anti-IntrinsicFactor] autoimmune destruction ➜ AMAG ➜ no IntrinsicFactor to facilitate {[Cobalamin VB12] terminal iLeum absorption} ➜ [mAcrocytic megaloblastic anemia]
dx = elevated Anti-IF
AMAG = Autoimmune Metaplastic Atrophic Gastritis
*HIGH YIELD*
Name the gastritis associated with pernicious anemia
_________________
What are the 3 main components of it’s MOD
AMAG = autoimmune destruction against intrinsic factor and oxyntic cells ➜ gastric fundus and gastric body destruction with:
- glandular atrophy
- intestinal metaplasia
- intestinal inflammation
_________________
AMAG = Autoimmune Metaplastic Atrophic Gastritis
What is Tumor Lysis Syndrome? (5)
{[cytotoxic chemotherapy] or [high grade lymphoma]} ➜ rapid lysis of neoplastic cells ➜”pt PUKED”
- Phosphate INC (⇪ [serum/urine P] binds [serum/urine Ca+] ➜ [⬇︎serum Ca+ and CaPhosphate renal stones])
- {Uric acid INC from pUrine DNA bases (serum and [urine ➜ Uric Acid stones]) = (px=allopurinol and IVF)} = DIAGNOSIS
- K+ INC ( ➜ arrhythmia)
- [Elevated (Ca+P & Uric Acid)renal stones ➜ AKI (IVF px)]
- [DNA base Purines] INC → Uric acid INC
Rasburicase
MOA?
[urate oxidase(not naturally in humans) recombinant] = catalyzes conversion of [INC Uric acid(i.e. from Tumor Lysis Syndrome)] ➜ [allantoin (excreted in urine)] for Tumor Lysis Syndrome px
_________________
Cancer pt on Rasburicase presents with AKI after starting chemo
Why is this?
Why didn’t Rasburicase stop it?
- Cancer = chemo ➜ [TLS PUKED] ➜ [Elevated (Ca+P & Uric Acid)renal stones ➜ AKI]
- Rasburicase only ⬇︎Uric Acid renal Stones. AKI can still be caused by Ca+P stones
Febuxostat
MOA
Allopurinol
MOA
Why is ChemoRadiation Therapy given together for Head/Neck CA versus Chemo or Radiation alone?
[60% Head/Neck CA are locally advanced at time of dx = inoperable] ➜ CRT as a duo INC the 5 year survival rate
Violent PCP should be treated immediately with ⬜ ⼀and Mild PCP should be treated with ⬜2
Violent PCP = [BENZO sedation]
Mild PCP = [low stimulation environment +/- benzo sedation]
***
[PCP (Phencyclidine)] is a [NMDA R Blocker hallucinogen]
How do you manage a patient using herbal medicine against medical advice (4)
- physician should*
1. explain risks of herbal preparations
2. document that you counseled patient to avoid herbal prep
3. document patient refusal/response to avoid herbal prep
4. follow patient closely for adverse effects
List the differences of [Peripherally Inserted central catheters (PICC)] as compared to [Centrally Inserted central catheters] (4)
PICC has [HIGHER UE DVT]
but…
PICC has lower
- infections
- procedural complications
- patient discomfort
Describe the following values for Iron Deficiency Anemia:
MCV
Iron
[Transferrin saturation]
TIBC
Ferritin
What is
TIBC
Total Iron Binding Capacity = TIBC = “Transferrin In Blood Calculation”
= [TOTAL amount of transferrin(transports iron) in serum]
(by calculation)
Describe the following values for Thalassemia:
MCV
Iron
[Transferrin saturation]
TIBC
Ferritin
Describe the following values for Anemia of Chronic Disease:
MCV
Iron
[Transferrin saturation]
TIBC
Ferritin
{ACD🔧: chronic/inflammation→IL6 secretion → [hepatic hepcidin secretion]→ [macrophage/intestinalferroportin]❌ from transfering Fe from FerriTin to Transferrin ➜ {[ ↧ Transferrin Saturation with an accompanying Transferrin “activity pause” = stops Transferrin from scavenging blood for more Fe → ⬇︎ Transferrin In Blood (⬇︎TIBC)] , [↥ FerriTin],} and ultimately [⬇︎Fe**in circulation*],
Describe the following values for Sideroblastic Anemia:
MCV
Iron
⬇︎MCV
⬆︎Iron
[4-allele deletionAlpha thalassemias] are characterized as ⬜ on electrophoresis
[HgbBarts tetramer of gamma (Hydrops Fetalis)]
Intrauterine death 2/2 CHF
What is [Hemoglobin SC] disease? (3)
- less severe variant of [Sickle Cell Disease (Hgb_SS & Hgb_SC)]
- ➜ mild normocytic anemia
- electrophoresis = [equal Hgb_S = Hgb_C]
Traditionally, FerriTin less than ⬜ indicates Iron Deficiency, but realistically why can this not be a hard rule?
[FerriTin < 15] = IDA
_________________
Most pts with [FerriTin 15-30] realistically are also iron deficient = Traditional rule can only rule IN IDA
Multiple Myeloma tx - 2
- [BorTezomib proteasome inhibitor]
OR
2.{[LD and if <70 yo(➜ BMT)}
[Lenalidomide + Dexamethoasone] –> [Bone Marrow Transplant]}
What is the issue with seeing IgG or IgA monoclonal “M” spikes on serum protein electrophoresis?
This does NOT automatically mean Multiple Myeloma. This can be seen in MGUS-Monoclonal Gammopathy of Unknown Significance which = common in older pts and only transforms to Multiple Myeloma 1%/year
Waldenstrom Macroglobulinemia MOD
________________
cp-5
Multiple Myeloma plasma cells overproduce IgM specifically –> hyperviscosity sx (HA, tinnitus)
- Neuropathy
- Engorged blood vessels
- HA
- tinnitus
- Raynaud Phenomenon
Waldenstrom Macroglobulinemia tx - 3
- PLASMAPHERESIS initially
- Chlorambucil + Prednisone = long term OR
- Fludarabine + Prednisone = long term
tx for Von Willebrand disease - 3
DDAVP desmopressin –(refractory)–> Factor 8 or vWF concentrate
DDAVP → ⇪Factor 8 → ⇪ vWF
(DDAVP releases subendothelial stores of vWF)
abx choice for [Acute Chest Syndrome in Sickle Cell Disease] -2
CefTriaxone (Strep Pneumo)
+
Azithromycin (Mycoplasma PNA)
treatment for iron overload in Hemochromatosis
therapeutic phlebotomy
Which CA presents as a pathologic lymph node of the head & neck in a smoker/EtOH patient?
metastatic SQC
MELAS stands for ⬜
Describe the clinical features (3)
MELAS
[Mitochondrial Encephalopathy + Lactic Acidosis + Stroke-like episodes]
_________________
- stroke-like episodes, lactic acidosis, seizures, m weakness, hearing loss
- onset LOE40 yo
- maternally transmitted only
SLE patients are at INC risk for developing which CA?
[Non-Hodgkin Lymphoma: (DLBL)]
Diffuse Large B-Cell
_________________
RAS_HH_ O_RR_ PAI_NN_
Clinical findings of Frostbite (6)
“Frostbite numbs your body, scars your body, then Killsyour body”
1st: numbs = [(Pallor +/- Thrombosis) + Anesthesia]
2nd: scars= [blister + eschar]
3rd: Kills = [Deep Tissue Necrosis, Mummification]
What is the treatment for Frostbite? (5)
In Frostbite, Thrombolysis is a treatment option
Initial treatment for Frostbite is ⬜. When is Thrombolysis considered to be used? (3)
[Rapid rewarming in water bath at 37-39C] ;
Thrombolysis is considered in patients who:
- have absent perfusion on angiography or technetium-99m scan
- amputation would be suboptimal
- seek care within 24h of injury (typically only used in these pts)
Malaria
Clinical features? (4)
- [FEVER + HA + THROMBOCYTOPENIA] in travelers returning from sub-Saharan Africa
- Dx = peripheral blood smear
- Plasmodium falciparum
- [Antimalarial prophylaxis before, during and after]
Although this herbal preparation has no clinically proven efficacy,
List the conditions it’s associated with treating:
* * *
Saw Palmetto
BPH
“GSG cause Bleeds”
Although this herbal preparation has no clinically proven efficacy
List the conditions it’s associated with treating:
* * *
garliC
HyperCholesterolemia
“[herbs GSG] cause Bleeds”
- Although this herbal preparation has no clinically proven efficacy,*
- List the conditions it’s associated with treating:*
- *
glucosamine
Osteoarthritis
- Although this herbal preparation has no clinically proven efficacy,*
- List the conditions it’s associated with treating:*
- *
chondroitin
Osteoarthritis
- Although this herbal preparation has no clinically proven efficacy,*
- List the conditions it’s associated with treating:*
- *
St.John’s wort
Depression
Chronic alcoholics Steal Phen-Phen & Never Refuse Greasy Carbs which keeps them UP
List the herbal preparations that cause bleeding (3)
“[herbs GSG] cause Bleeds”
-Ginkgo biloba
-Saw Palmetto
-Garlic
CYanide is released during the combustion of items containing the compounds [⬜ and ⬜]. Give 2 examples of materials that contain both these compounds
Carbon & Nitrogen ; PLASTIC | Polyurethane foam
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
Describe why patients with CYanide poisoning have [bright CHERRYRed skin]
[CYanide(released from burning PLASTIC|using NitroprussideRx)] inhibits [mitochondrial oxidative phosphorylation] ➜ forces cell to switch to anaerobic metabolism ➜ lactic acid formation = [lactic acidmetabolic acidosis].
unused arterial oxygen remains in blood ➜ unused venous oxygen = [bright CHERRYRed skin]
_________________
tx = [Hydroxocobalamin +/- Sodium thiosulfate]
{CYanide tox sx = [bright CHERRYRed skin], Yucky bitter almond breath, brain⇪*seizure*, brain⬇︎*CNS depression* , [lung⇪ f/b lung⬇︎][tachypnea f/b bradypnea]
Both CYanide and Carbon MONOxide poisoning cause bright Red Skin
How can you differentiate the two? (2)
- [CYanide (released from burning PLASTIC)] inhibits [mitochondrial oxidative phosphorylation] vs [CO binds hgb with higher affinity than O2 ➜ DEC oxygen delivery]
- [CO = Carboxyhemoglobin > 25%] vs [CYanide = nml Carboxyhemoglobin]
_________________
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
Sx of CYanide poisoning (6)
- Bright CHERRY Red Skin (also seen in CO poisoning)
- Yucky Bitter Almond breath
- brain⇪seizure
- brain⬇︎CNS depression
- [lung⇪ f/b lung⬇︎][tachypnea ➜ bradypnea]
- [lactic metabolic acidosis]
🧠inhibits oxidative phosphorylation → anaerobic metabolism → lacticMA
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
Why do pts receiving Nitroprusside have INC risk for ⬜ toxicity? (2)
CYanide;
▶Nitroprusside (rapid short acting vasoDilator) metabolizes into both:
- Nitric Oxide ➜ vasoDilation
- CYanide ➜ converted to thioCYanate ➜ RENAL excreted
_________________
▶Patients with renal insufficiency = unable to excrete thioCYanate ➜ CYanide toxicity
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
What’s the most common inherited thrombophilia?
Factor 5 Leiden
Describe the order of events for ordering blood transfusion for a patient (4)
blood is ordered for transfusion:
Type Serum Carefully, Dude
1st: Type: [Recipient patient’s] blood is typed = ABO and Rh determined
_________________
2nd: [Screen (PTAS)]: Recipient’s serum Screened for [unexpected preexisting autoantibodies (previously made against RBC antigens E, L or K from previous transfusions)] = [PreTransfusion Ab Screen]
_________________
3rd: Crossmatch Recipient’s blood with [blood they will be receiving] in-vitro first
_________________
4th: Decide: [✅Transfuse (⊕unexpected e/l/kRBC AutoAntibodies or ⊕Crossmatch rejection?)🚫Transfuse ➜ Identify Ab]
[GVHD-Graft Versus Host Disease] is common after _____ or _____ transplant
_________________
It involves [Graft_Donor ⬜ cells] attacking which 3 parts of the [Host_Receipient body]?
Bone marrow; Organ ;
_________________
T ;
- Skin
- Liver
- GI
This occurs because [Graft_Donor] T-cells recognize major and minor HLA antigens of the [Host_Recipient Skin//Liver/GI]
What is the px for [Febrile nonhemolytic transfusion reaction]
Leukoreduction of [graft_donor] blood
_________________
this will also ⬇︎ risk of HLA alloimmunization and CMV transmission
What is Leukoreduction used for? (3)
[Leukoreduction of [graft_donor] blood]
will ⬇︎ risk of:
1.[Febrile nonhemolytic transfusion reaction],
2.[HLA alloimmunization]✳
3.CMV transmission(CMV resides in Leukocytes)
✳alloimmunization: = *host_recipient immune response to otherwise [foreign antigens (blood group ag|histocompatibility ag) from ANOTHER PERSON] [⼀usually from incidental exposure like transfusion] *
A patient receiving Nitroprusside for Aortic Dissection develops acute metabolic acidosis and confusion.
Diagnosis?
Cyanide toxicity
{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}
The 3 phases of [Primary Hemostasis platelet plugging] are ____
Describe the 3 phases of [Primary Hemostasis platelet plugging (1HS)] that stops the bleeding after you cut your arm
“superficial cut using DAG on it !”
1st: [aDherence] of circulating [inactivated platelet’s Gp1B] to exposed vWF of subendothelial collagen
→2nd: [aActivation & secretion] = Activated platelet → secretes fibrinogen and {[ThromboxaneA2 & ADP] → [INC (Gp2b/3a_platelet fibrinogen R) expression on platelets]}
→ 3rd: [aGgregation] of platelets via fibrinogen binding them together via [Gp2b/3a_platelet fibrinogen R]
= [weak 1º PLATELET PLUG]
______________________
How does Chronic Kidney Disease cause [mucosal bleeding1HS❌]?
__________________
1HS❌: [Primary Hemostasis platelet plugging disruption ]
patients with [Chronic Kidney Disease] have INC ____ dysfunction → what clinical presentation? (5)
platelet;
- CKD patient
- [1HS❌ (⇪ mucosal bleeding/bruising)]
- [platelet count nl]
- [PT/INR nl]
- [aPTT nl]
__________________
1HS: [Primary Hemostasis platelet plugging]
a. [Chronic Kidney Disease] causes dysfunction of [____ cells] which → ________
b. How does this happen?
a. platelet; [1HS❌ (⇪ mucosal bleeding/bruising)]
__________________
1HS: [Primary Hemostasis platelet plugging]
a. [Chronic Kidney Disease] causes dysfunction of [____ cells] which → ________
* * *
c. Treatment? (2)
a. platelet; [1HS❌ (⇪ mucosal bleeding/bruising)]
* * *
c.
- Sx = [desmopressin dDAVP (INC vWF secretion from endothelial cell)]IV or SQ
- Asx = observe
__________________
1HS: [Primary Hemostasis platelet plugging]
a. [Gp2b/3A receptor] is found on the ___ cell, is called ___ when deficient, and is responsible for what?
* * *
b. Name the [Gp2b/3A R Blockers] (3)
a. platelet;
[glanzmann thrombasthenia] ;
aGgregates platelets (—by binding [Activated platelet Gp2b/3A receptor] with a another [Activated platelet Gp2b/3A receptor] via fibrinogen)
b. “he ATE my Gp2b/3A”:
[Abciximab, Tirofiban, Eptifibatide]
__________________
[Gp1b receptor] is found on the ___ cell, is is called ___ when deficient, and is responsible for what?
a. platelet;
[Bernard-Soulier syndrome] ;
[aDheres [inactivated circulating platelets] to vWF (which will already be bound to exposed subendothelial collagen)
→platelet [Activation & secretion(of “F.A.T.” stuff)] → platelet aGgregation = [DAG 1HS]
In addition to analgesia, why is ASA also considered a blood thinner? (2)
ASA inhibits COX
✏️ { [platelet Arachidonic Acid] —(via COX)-→ converted to [TXA2 (thromboxane)] → [platelet TXA2 (ADP also)] INC expression of [Gp2b/3A_platelet fibrinogen R] which enables→ [platelet aGgregation] }
✏️ASA inhibits COX → No [TXA2 (thromboxane)]= No platelet aGgregation = No Clotting (“thins” your blood)
Which bleeding reversal agent should be used to treat DIC?
ffp
DVTs can either be primary or secondary
Describe what the main differences of secondary DVT are (4)
- reversible or
- time limited
- (ex: surgery, pregnancy, OCP, trauma)
- [Warfarin tx for only 3-6 months ( ≥6 mo for [1° idiopathic])]
What is the symptom triad for acute opioid intoxication
- depressed brain,* [= Somnolent/AMS]
- depressed blacks,* [“-pupils” = miosis])
- depressed breath* [= shallow bradypnea ≤12 RR]
Organophosphates MOA = ⬜
Where are they found? (2)
[potent inhibitors of AChE]
→ [No ACh–breakdown] → [ACh accumulation] → hyperactivation of mCN receptors =
{[“OTC dUMBBELLS Create Muscle”] ? …….PAID}
[agricultural pesticides], [sarin(WARFARE AGENT)]
_________________
mCN: muscarinic | CNS | Nicotinic
Organophosphates are potent inhibitors of ⬜ which leads to ⬜
Sx (11)
AChE ;
[ACh accumulation] → {[“OTC dUMBBELLS Create Muscle”] ? …….PAID}
[“OTC dUMBBELLS Create Muscle”]
Organophosphate _T_oxic Cholinergic :
- [diarrhea/Urination/Miosis/Bradycardia/Bronchospasm( → Respiratory failure) /Emesis/Liquid lung bronchorrhea /Lacrimation/Salivation]muscarinic
- [Coma/Seizure]CNS
- [Muscle(weakness/paralysis/fasciculation)]Nicotinic
Organophosphates are potent inhibitors of ⬜ which leads to ⬜
Treatment (4)
AChE ;
[ACh accumulation] → {[“OTC dUMBBELLS Create Muscle”] ? …….PAID}
PAID
- [Pralidoxime ⼀reactivates AChE]
- [Atropine anticholinergic⼀ competitively inhibits ACh]
- [Intubation prn + ABCs (give activated charcoal if exposure ≤1h)]
- [Decontamination]
cp for [Niacin B3] deficiency (4)
DDDD(AKA Pellagra)
Dementia
[Dry mouthSTOMATITIS / CHEILOSIS]
Diarrhea
[Dermatitissymmetrical/blister vesicles in sunexposed areas]
💡Niacin uses Tryptophan for synthesis, & CaRcinoid tumors do also
Radionuclide bone scans detect ⬜, which makes it sensitive for detecting [⬜ osteoLytic | osteoBLASTIC] bone metastasis (from ⬜ )
areas of INC bone turnover;
osteoBLASTIC(prostate/SOLC/Hodgkin)
Name the Cancers that metastasize into [osteoBLASTIC bone lesions] (3)
- Pt with osteoBLASTIC CA c/o dull shoulder pain, worse at night*
How do you work them up for potential bone metastasis? (4)
Prostate / SOLC / Hodgkin
Name the Cancers that metastasize into [osteoLytic bone lesions] (3)
- Pt with osteoLytic CA c/o dull shoulder pain, worse at night*
How do you work them up for potential bone metastasis? (3)
- nMn*
- N*onSOLC / Multiple Myeloma / NonHodgkin
- *
Name the Cancer that metastasizes to form [mixedosteoBLASTIC or osteoLytic bone lesions]
- Pt with mixed osteoB/L CA c/o dull shoulder pain, worse at night*
How do you work them up for potential bone metastasis? (5)
BREAST
Tx for Sickle Cell Pain Crisis - 4
1.Dehydration = [IV Hydration + IV Analgesia]
_________________
2.[Infxn (⊕fever|⊕WBC)] =cefTriaxone
_________________
3.Cold🌡=[Transfusion(if ⊕CVA|Retinal infarct|aCS| Priapism)
_________________
4.“Kan’t breathe_hypoxia” = [O2 + Hydoxyurea to carry more O2⇪HbF]
treat the 4 SCD triggers (DICK)
aCS = acute Chest Syndrome
Tx for Hereditary Spherocytosis - 2
- [Folic acid B9] chronically
- Splenectomy (stops hemolysis)
When is it ok to give Platelet transfusion to HUS|TTP?
NEVER!!!
give plasma EXCHANGETTP tx|supportiveHUS tx
TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → FMNRT TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ (TTP Tx = EXCHANGE plasma containing anti-A13vM or deficiency of A13vM for normal plasma)
What is the most common adverse reaction to blood transfusion?
What causes it?
When does it present?
1-6h[Febrile Nonhemolytic transfusion reaction] = F/C from cytokine accumulation during blood storage]
Autoimmune Hemolytic Anemia and Hereditary Spherocytosis BOTH can cause extravascular hemolytic anemia
How can you discern the two? - 2
[DAT] and [fam hx]
AIHA = [⊕DAT] (negative fam hx)
_________________
Hereditary Spherocytosis = [⊝DAT] (positive fam hx)
- DAT* = [Direct(Coombs) AntiHumanGlobulin Test]
What is the purpose of the
[Indirect_Coombs AntiHumanglobulin Test (IAT)]
_________________
Explain IAT MOA (2)
IAT = [Indirect_Coombs AntiHumanglobulin Test] =
▶Uses [IAT and test blood] in lab to determine if h_r serum contain [preformed IsoAgglutinins(IgM>IgG)] that agglutinate to common RBC antigens = “Antibody Screen”
▶Indirectly In Vitro: Mixes [test blood(which contain RBC loaded with common RBC antigens)] with host_recipient’s [plasma w/antibodies]. ⼀ The IAT will bind to any {h_r [Ab|compliment (IgM/IgG/C3d/C3/etc.)} that have inappropriately agglutinated to RBC common antigens→ IAT is visualized to identify the RBC antigens (if any) h_r Ab are sensitized to.
[IAT = Indirect CAT]:
*CAT: Coombs AntiHumanglobulin Test] // [g_d: graft_donor] // [h_r: host_recipient]
What is the purpose of the
[Direct_Coombs AntiHumanglobulin Test (DAT)]
_________________
Explain the DATMOA (3)
DAT = [Direct_Coombs AntiHumanglobulin Test] =
[CAT: Coombs AntiHumanglobulin Test] // [gd\ : graft_donor] // [hr: host_recipient]
_________________
DAT
▶Uses CAT to determine if [gd</sup>RBC] are ACTIVELY being Sensitized (and ultimately hemolyzed) by [Transfusion/Drug-Induced/ hr</sup>IgM]
▶DIRECTLY EN VIVO: host_recipient blood is taken and given CAT DIRECTLY → which will bind to (and allow us to visualize/identify) any {[hr\Ab|compliment (IgM>>IgG/C3d/C3/etc)] ACTIVELY agglutinating gd\RBC}
▶[⊕DAT is ALWAYS ABNORMAL] and usually comes from [host_recipient IgM and compliment] (</sup>but incomplete DAT comes from IgG | compliment acting alone)*
What conditions would you expect to find a
positive [Direct_Coombs AntiHumanglobulin Test (DAT)] (5)
DAT = [Direct_Coombs AntiHumanglobulin Test] =
[CAT: Coombs AntiHumanglobulin Test] // [gd\ : graft_donor] // [hr: host_recipient]
_________________
[⊕DAT occurs in ___]
- [Acute Intravascular Transfusion Hemolysis]
- [Delayed IgG EXtravascular Transfusion Hemolysis]
- [Drug-Induced IgG EXtravascular Transfusion Hemolysis]
- [Autoimmune Hemolysis]
- [HDN (Hemolytic Disease of Newborn)]
During Blood Transfusion both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes
How do you differentiate the two based on:
Onset
Cause
≤minutes[Anaphylaxis (IgA-deficient pts’ anti-IgA antibodies attack donor IgA)] = [Respiratory Distress, Angioedema]tx = Epi, antihistamine, CTS, [CardioPulm support]
* * *
≤1 hr[Acute Hemolytic Reaction (ABO mismatch 2/2 clerical error vs chronic transfusions) → [Preformed IgM using compliment (will have ⊕DAT)] → Intravascular hemolysis ] = [Tachycardia/LumbarPain/Hgbnuria/Bleeding_hypOtension_DIC/ChestConstriction/FluLikeSx]tx = [NORMAL SALINE IVF]
During Blood Transfusion both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes
How do you differentiate the two based on:
- Sx*
- Tx*
≤minutes[Anaphylaxis (IgA-deficient pts’ anti-IgA antibodies attack donor IgA)] =
[Respiratory Distress, Angioedema]tx = Epi, antihistamine, CTS, [CardioPulm support]
- ≤1 hr*[Acute Hemolytic Reaction (ABO mismatch 2/2 clerical error vs chronic transfusions) → [Preformed IgM using compliment (will have ⊕DAT)] → Intravascular hemolysis ] =
[Tachycardia/LumbarPain/Hgbnuria/Bleeding_hypOtension_DIC/ChestConstriction/FluLikeSx]tx = [NORMAL SALINE IVF]
[T or F]
[Premedication with antihistamines and APAP] can prevent blood transfusion reactions
FALSE
[CLL-SLL] is a ⬜ type of CA , associated with 4 sx ( ⬜4), all to which indicate a worse prognosis
How is [CLL-SLL] diagnosis confirmed? (2)
_________________
CLL-SLL: Chronic Lymphocytic Leukemia-Small Lymphocytic Lymphoma
[monoclonal B-lymphocyte leukemia]; LATO sx
LAD
Anemia
Thrombocytopenia
OrganomegalyLiver/Spleen
[smudge cells(peripheral smear)] and [flow cytometry]
What should you remember regarding Biopsying Metastatic Cancer ? (4)
Patients with
▨[limited CA: solely Organ/Organ’s lymph nodes] → limited bxorgan or organ LN]
▩ [EXTENSIVE CA: metastasis/involves supraclavicular LN → EXTENDED bxof one of the metastasized sites instead [(supraclavicular (if involved)] > other sites]
= this is because metastasized sites (like Supraclavicular LN excisional bx) tend to be ⬇︎invasive and ⬇︎complications for biopsy
_________________
ex: Pts with suspected metastatic Lung cancer (if possible)should have initial bx from one of the metastatic sites(SCLV-if involved) > other
Vancomycin Infusion Reaction
[NON-IgE] drug rxn involving vancomycin binding directly/activating mast cells → histamine release → [diffuse pruritus, erythema] ⼀proportional to infusion rate and resolved with d/c
CO binds with greater affinity than O2 to Hgb and interferes with O2 offloading
Describe the Symptom course for carbon Monoxide toxicity (9)
sx: “Monixide hurts my [MIND’S → SSSH]
Malaise
Irregularly SHARED HEADACHE (w/roommate, housemate, etc)
Nausea
Dizziness
Skin ∆ [PINK-like cYanide tox] or [BLUE-like methemoglobinemia tox]
* * *
Seizure/Syncope/Sleepy-COMA/Heart❌
tx: high-flow O2 via nonrebreather → hyperbaric oxygen
tx for Carbon Monoxide toxicity (2)
_________________
sx: “Monoxide hurts my [MIND’S → SSSH
[high flow O2 via nonrebreather] –(if severe)–> hyperbaric oxygen
Name the major causes of Carbon Monoxide toxicity (3)
_________________
What are 2 crucial diagnostics for Carbon Monoxide toxicity
_________________
sx: “Monoxide hurts my [MIND’S → SSSH]”
scombroid poisoning
a. etiology
* * *
b. symptoms (6)
a. *(often confused for allergic rxn) </sup>*in seafood stored at temperatures > 15C, histidine undergoes decarboxylation → histaM**ine =
b. [flushing |throbbing HA|palpitations|abd cramps|diarrhea|(oral burning +/- “bitter taste”)] 10-30m after ingesting (usually)FISH
Pufferfish Poisoning
symptoms (3)
*perioral tingling
*paralysis
*poor coordination
clinical features of Serum Sickness (3)
- [type 3 immune complex-mediated hypersensitivity rxn]
- primarily occurs after receiving [antibodiesMonoclonal/Chimeric] or [antitoxinsheterologous]
- SSLR = gzd urticaria + feverlow grade + arthralgia + LAD
_________________
SSLR: Serum Sickness-Like Reaction
Tx for [1º myelofibrosis (CIPM)]
_________________
CIPM: [Chronic Idiopathic Primary Myelofibrosis]
{ allogenic hematopoietic stem cell transplant < [60 Y/O]< palliative care }
Acute Leukemia will present with signs of _____
Which acute leukemia is associated with [Auer rods (eosinophilic inclusions)]?
PANcytopenia ; [aPL ⼀acute PROMyelocytic Leukemia M3 (T15/17)]
Dx = smear showing [myeloblast>20%] –> flow cytometry for confirmation
What are the toxicities for MTX?
Octreotide is a ⬜ used to treat ⬜
________________
explain how
[somatostatin 14 analogue] ; [Somatotrope - Functional Pituitary Adenoma]
________________
inhibits [pituitary somatotrope] from releasing Growth Hormone in a [functional pituitary adenoma]
By 21 years old, a fully immunized patient should have ⬜ total [TETANUS toxoid vaccines] .
Name the vaccines and what age they’re given
6 = (this is the expected # lifetime Tetanus vaccines)
#5 if 18 yo
________________
[DTaP = (2 / 4 / 6 / 15 ) months old]
+
[TDaP at 11 years old]
+ TDaP booster @ 21 years old
⬜ is the GREATEST risk factor for Male Breast Cancer.
⬜ is the second greatest risk factor for Male Breast Cancer.
And ⬜ is the third greatest risk factor for Male Breast Cancer
________________
Etx for Greatest risk factor? ; etx for 2nd greatest risk factor?
BRCA mutation > > > Klinefelter Syndrome
> [LAME (Liver failure/(Always Eating {Obese})/Marijuana/(Estrogen:androgen ratio ⇪ {gynecomastia})]
________________
BRCA = auto DOM mutations ➜ [⇪ Male Breast CA risk x 100]
[klinefelter syndrome XXY] = male having extra “X” chromo ➜ [Estrogen:androgen ratio ⇪] ➜ [⇪ Male Breast CA risk x 20]
normal blood glucose is ⬜
What is Whipple’s triad and what does it indicate?
60-100
(some can go down to 45 with no sx)
________________
Whipples = [low BG] + [PUSH low BG sx] + [PUSH low BG sx improve after glucose administration] = true hypOglycemia
________________
hypOglycemia sx = need PISH juice = Palpitations/Uneasy Irritable/Sweating/HA
how do you prevent Tumor Lysis Syndrome? - 2
________________
how do you treat Tumor Lysis Syndrome (with AKI) -2?
________________
Why do these differ?
AF ➜ RF
px = AF: [Allopurinol (xanthine oxidase inhibitor)] +Fluids IV]
________________
TX (for AKI 2/2 TLS) = RF: [Rasburicase (urate oxidase analogue)] + Fluids IV]
________________
[Allopurinol prevents Additional serum uric acid formation] whilst [Rasburicase metabolizes Realtime (already existing) serum uric acid]
the presence of [HbA 60% : HbS 40%] on electrophoresis is c/w ⬜ .
What are the subsequent sx of this?
Sickle Cell TRAIT = ASYMPTOMATIC (does not cause Anemia)
Anemia of Chronic Disease
MOD (5)
⭐caused by IL6 chronic inflammation → liver secretes hepcidin → [blocks 🆚 suspend] {[DJ enterocyte ferroportin] and [macrophage ferroportin]} from transfering iron from FerriTinstorage to Transferrintransport
⭐(→🔻TS(if ferroportin _blocked_) 🆚 normal TS(if ferroportin only _suspended_)),
⭐and exact opposite for FerriTin= 🔺*(ferroportin blocked) * 🆚 nml(ferroportin suspended FerriTin
⭐but additionally❗️ all this hoopla also paradoxically keeps Transferrin out of circulation (which → ⬇︎TIBC (but with 🔻/normalTransferrin Saturation)) = rare*
⭐AND ULTIMATELY this ➜ {[(Normoinitially→microlater)cytic] normochromic anemia}
*Typically Transferrin Saturation and TIBC are inverse related
so….
_________=________
iron studies ={[↧Transferrin Saturation with paradoxic ⬇︎TIBC]*,
[↥FerriTin],
[⬇︎ironin circulationiron in circulation is low because it’s still being sequestered to FerriTin by Hepcidin ]} =
{[🢗MCVNormo –(to)–> micro -CYTIC ] [NormoCHROMIC] }
___________________________x____________________________________
🔎DJ = Duodenal-Jejunal
🔎↧ = (DEC or normal)
🔎↥ = (INC or normal)
🔎TS = Transferrin Saturation
Why are abx NOT used in treating Hemolytic Uremic Syndrome?
________________
What is the management for Hemolytic Uremic Syndrome?
HUS HAT
killing bacteria could ➜ ⇪ release of Shiga toxin
________________
SUPPORTIVE CARE ONLY
(fluid/electrolyte mgmt | blood transfusions | dialysis)
features of Multiple Myeloma -4
osteoclast activating factor ➜CUBPsx
1.[(CRAB) - end organ damage]
hypercalcemia(→ vertebral dark lytic lesions/bone fx, constipation, depression)
Renal failure(2/2 Ig and Bence Jones proteinuria –> DEATH)
Anemia normocytic
[Back pain i\ vertebral dark lytic lesions and bone fx]</sub>
________________
2.[Urine IgG or Urine IgA]
________________
3.[Bone marrow with ≥10% clonal plasmacytosis/plasmacytoma (If Infection → DEATH!)]
________________
4.[Protein (M Protein) in serum]
[CRYOGLOBULINEMIA TYPE 2] MOD
[Chronic Viremia/Autoimmune disease] ➜ [B cell hyperactivation] ➜ forms IgM which bind to IgG = ⇪ mixed circulating immune complexes
these [circulating immune complexes] desposit in small vessels ➜ INFLAMMATORY vasculitis (glomerulonephritis)
________________
What 3 physical exam findings indicate widespread microvascular occlusion?
- Livedo reticularis
- digital ischemia
- retiform purpura (net like reflect of vasculature - image)
[Febrile nonhemolytic transfusion rxn] occurs ⬜ after transfusion starts. and Pts have what sx -3?
_________________
How is this related to Leukoreduction? -3
[1H-6H] ; [Fever / Chills / Malaise] (NO HEMOLYSIS)
_________________
Leukoreduction =
⬇︎[Febrile nonhemolytic transfusion rxn]
⬇︎HLA alloimmunization✳
[⬇︎CMV transmission (since CMV resides in Leukocytes)]
✳alloimmunization: = *host_recipient immune response to otherwise [foreign antigens (blood group ag|histocompatibility ag) from ANOTHER PERSON] [⼀usually from incidental exposure like transfusion] *
[Glucose-6-Phosphate Dehydrogenase] deficiency
cp -3
[HEMOLYSIS WITH UNCONJUGATED HYPERBILIRUBINEMIA → JAUNDICE]
[SUDDEN BACK PAIN]
FATIGUE
________________
X-linked RBC G6PD defect in African/Middle Eastern/Southeast asian ➜ ⇪ RBC hemolysis from [DASANI H2O2 oxidant stress]
[Glucose-6-Phosphate Dehydrogenase] deficiency
etx -5
✏️ [X-linked RBC (G6PD_Glutathione reductase) deficiency] in African/Middle E/SE asian
✏️= [“DASANI” H2O2 oxidant stress] → [RBC H2O2 ⇪ (since there’s no G6PD to drive GSH buffer rxn which normally takes up oxidant stress)] → [Oxidation denaturation of Hgb Sulfhydryl groups] = Formation of Heinz bodies in RBC→ [RBC damage and ⬇︎ membrane flexibility] = sx :
⬇︎
✏️a.. 🅸1 = Intravascular hemolysis from [Oxidized denatured Hgb Sulfhydryl group membrane damage] → *[HEMOLYSIS WITH UNCONJUGATED HYPERBILIRUBINEMIA → JAUNDICE]**
✏️b.🅸2: [Heinz body RBC(RBC with oxidized denatured Hgb Sulfhydryl groups)] travel thru splenic cord and splenic macrophages BITE out Heinz body] → [Bite RBC] –(eventually after enough bites) forms into→unstable Spherocyte} → 🅸
✏️c. 🄴Xtravascular hemolysis from [Heinz body-RBC] suddenly getting stuck in spleen → removed by splenic macrophages → [SUDDEN BACK PAIN] + FATIGUE
*🔎 🅸 = Intravascular hemolysis from membrane damage → [HEMOLYSIS WITH UNCONJUGATED HYPERBILIRUBINEMIA → JAUNDICE]**
[Glucose-6-Phosphate Dehydrogenase] deficiency
How do you diagnose this? -3
[X-linked RBC (G6PD_Glutathione reductase) defect] in African/Middle E/SE asian
1.[measure G6PD activity]
2.[peripheral smear:bite RBCs]
3.[peripheral smear:[heinz body RBCs] (= accumulated “SHODE =byproducts of INC H2O2 on RBC) ]
SHODE: [Sulfhydryl Hgb ⼀ Oxidized, Denatured entities]
diagnosis?
[Heinz body RBCs(accumulated oxidized denatured hgb sulfhydryl groups)] in G6PD
GLUCOSE 6 PHOSPHATE DEFICIENCY
Diagnosis? | What’s the best diagnostic test for this condition?
Spherocytes(Hereditary Spherocytosis | G6PD)
_________________
Osmotic Fragility test
cp for Thalassemia trait -5
_________________
How do you differentiate α from β Thalassemia?
- anemia that is
- ⼀microcytic (low mean corpuscular volume)
- ⼀hypOchromic (low mean corpuscular hgb)
- [⇪ ferriTin (INC RBC turnover ➜ more iron to Tuck into storage)]
- [normal RDW (all RBC uniformly made microcytic)]
_________________
Hgb electrophoresis: [βT minor = ⇪ HgbA2]
[Gp2b/3A R blockers] are anti-⬜
________________
Name all 3
anti-platelet
________________
“I ATE [Gp2b/3A] for breakfast”
[ABCiximab / Tirofiban / EpTiFibatide]
Describe Management for Tetanus Prophylaxis
What’s the 1ST medication given for Atheroslcerosis?
_________________
When is it actually indicated to give? (3)
STATIN
_________________
- [LDL ≥190]
- [age ≥40 + DM]
- [(10y ASCVD risk) ≥7.5%]
________________
THIS IS REGARDLESS OF BASELINE LDL
Chronic Granulomatous Disease MOD (3)
▶[X-linked recessive 1° immunodeficiency]
▶defect in [neutrophil (NADPH oxidaseresp. burst)] ➜ impairs neutrophil superoxide formation ➜ [impaired neutrophil intracellular killing(neutrophils can eat BUT NOT KILL)]
▶ = defective granuloma formation ➜ recurrent [catalase positive infectionsfrom “SPACE Bugs”]
(Aspergillus = MAJOR COD / Staph A=liver/skin abscess/adenitis)
▶*(catalase+) infections = SPACE BugsStaphA&Serratia/Pseudomonas/Aspergillus&Nocardia/Candida/Enterobacter/Burkholderia-cepacia</sup*
▶dx = nitroblue tetrazolium test</sub>
Chronic Granulomatous Disease
management? (3)
[(SMXTMP) + itraconazole]px
_________________
[interferon-2]tx
▶(catalase+) infections = SPACE BugsStaphA&Serratia/Pseudomonas/Aspergillus&Nocardia/Candida/Enterobacter/Burkholderia-cepacia
▶dx = nitroblue tetrazolium test
Name the most common [Catalase POSITIVE] organisms (5)
_________________
Which 2 are related to Chronic Granulomatous Disease?
and how?
▶*(catalase+) infections = SPACE BugsStaphA&Serratia/Pseudomonas/Aspergillus&Nocardia/Candida/Enterobacter/Burkholderia-cepacia</sup*
_________________
[Chronic Granulomatous Disease]
➜ (Aspergillus_ = MAJOR COD)
➜ (StaphA_=liver/skin abscess/adenitis)
dx = nitroblue tetrazolium test
Describe the process of EtOH breakdown to Acetic Acid and explain how Metronidazole disrupts this
Metronidazole has Disulfiram-like activity –> (toxic)Acetaldehyde accumulation –> Flushing/NV/Cramps after drinking
Describe pathophysiology for acute APAP OD
_________________
Describe the 4 clinical stages of APAP OD
How do you diagnose APAP OD? (2)
_________________
⬜ and [N-AcetylCysteine] are the 2 mainstays of treatment. When is NAC most effective?
DELAYED HOSPITAL PRESENTATION = WORST OUTCOME
[Activated Charcoal (if within 4 h post ingestion)]
NAC is most effective when given within 8 hours post ingestion prior to hepatoxicity
MOD for APAP overdose
_________________
⬜ and [N-acetylcysteine] are mainstay treatments, but NAC is most effective if given ⬜. How does NAC work?
_________________
DELAYED HOSPITAL PRESENTATION = WORST OUTCOME
during APAP OD,
APAP –(via CYP2E1)–> [toxic NAPQI] and [toxic NAPQI] depletes intrahepatic glutathione as it’s ➜ [NON-TOXIC cysteine & mercapturic acid]
once intrahepatic glutathione is all depleted [toxic NAPQI] accumulates ➜ hepatotoxicity
_________________
[Activated Charcoal (if within 4h post ingestion)]- binds APAP
[NAC (most effective within 8 hrs post ingestion)] ⇪ intrahepatic glutathione and restores [(toxic NAPQI) ➜ (nontoxic cysteine & mercapturic acid)]
_________________
protective < [EtOH chronicity] < exacerbant
MOD for APAP overdose
_________________
How does EtOH affect this process? (2)
DELAYED HOSPITAL PRESENTATION = WORST OUTCOME
during APAP OD,
APAP –(via CYP2E1)–> [toxic NAPQI] and [toxic NAPQI] depletes intrahepatic glutathione as it’s ➜ [NON-TOXIC cysteine & mercapturic acid]
once intrahepatic glutathione is all depleted [toxic NAPQI] accumulates ➜ hepatotoxicity
_________________
acute EtOH competes for CYP2E1 ➜ DEC [toxic NAPQI] = protective
CHRONIC EtOH upregulates CYP2E1 ➜ INC [toxic NAPQI] = exacerbant
_________________
[Activated Charcoal (if within 4h post ingestion)]- binds APAP
[NAC (most effective within 8 hrs post APAP ingestion)] ⇪ intrahepatic glutathione and restores [(toxic NAPQI) ➜ (nontoxic cysteine & mercapturic acid)]
iron deficiency anemia and thalassemia both cause microcytic anemia
What’s a labatory method for differentiating them?
MentzeR Index = MCV/RBC
thalassemia < [MentzeR Index of 13] < IRON DEFICIENCY ANEMIA
_________________
Why does Carcinoid Syndrome cause Niacin B3 deficiency?
Carcinoid tumors utilize Tryptophan to secrete tons of Serotonin. Tryptophan is also needed to make Niacin B3. This can –> Pellagra DDDD
Do not confuse Carcinoid Syndrome with VIPoma which presents similarly but VIPoma affects Pancreas while Carcinoid affects small intestine
cp for carbon Monoxide poisoning?-9
________________
Dx?
sx: “Monixide hurts my [MIND’S → SSSH]
Malaise
Irregularly SHARED HEADACHE (w/roommate, housemate, etc)
Nausea
Dizziness
Skin ∆ [PINK-like cYanide tox] or [BLUE-like methemoglobinemia tox]
* * *
Seizure/Syncope/Sleepy-COMA/Heart❌
________________
Dx = [ABG Carboxyhemoglobin levels]
⚠️ Monoxide tox can present very similarly to [cYanide tox (which is also [PINK SKIN BUT with Yucky bitter almond breath)] and [Methemoglobinemia tox (BLUE SKIN)] so be careful!
toxicity of what 2 substances causes PINK SKIN?
{carbon Monoxide tox(MIND’S → SSSH) ➜ [PINK SKIN]}
________________
{CYanide tox ➜ [PINK SKIN with Yucky BITTER ALMOND BREATH]}
Antidote for CYanide toxicity - 4
“cYaMonoxide toxicity Needs Overt Smoke Help”
empiric tx for cYanide and (carbon)Monoxide toxicity
- [Nitrites(induces methemoglobinemia)]
- Oxygen 100% (CO tx)
- [Sodium thiosulfate]
- [HydroxoCoBalamin(binds cYanide ➜ excretable cYanocobalamin)]
________________
empircally treat smoke inhalation pts for CYanide tox!
[SEVERE <28C (or unresponsive Moderate) HYPOTHERMIA]
management
_________________
sx? (3)
[SEVERE <28C (or unresponsive Moderate)]:
[ACTIVE CORE REWARMING (WARM PERITONEAL LAVAGE, WARM HUMIDIFIED O2)]
_________________
{“(Cut ) BLM!”–tx–> [ACTIVE CORE🔥]}
BLM = Brain/Liver&heart/Muscle
{“(Cut) BLM”–tx–> [ACTIVE CORE🔥]} =
🧠 Brain= _C_OMA
🫁Lungs/heart = _C_ardiac Vt arrhythmia
💪Muscle = _C_V Collapse
[mild 32-35C hypOthermia]
management
_________________
sx? (5)
[mild 32-35C hypOthermia]:
[passive external warming ( warm blankets & remove wet clothes)]
_________________
{“(FAST) BLM”–tx–> [passive🅇🔥]}
BLM = Brain, [Lungs&heart, Muscle] || 🅇=E🅇ternal
{“(FAST) BLM”–tx–> [passive🅇🔥]} =
🧠 Brainataxia, dysarthria
🫁Lungs/hearttachypnea/tachycardia
💪MuscleINC shivering
a. tPA MOA (3)
_________________
b. What other 2 agents have same MOA?
c. Which agent has opposite MOA?
b. Streptokinase , Urokinase
c. Aminocaproic acid
What is the treatment for ASA toxicity? (2)
_________________
MOA for this tx?
[NaHCO3 IV] ➜ [HemoDialysis (if severe)]
_________________
[NaHCO3 IV] Alkalinizes Blood and Urine ➜ basic blood/urine environment ➜ deprotonation of Salicylates ➜[Salicylate ion] inability to be reabsorbed into CNS or into [blood from renal tubules] ➜ [⇪ salicylate ion excretion]
severe = cerebral/pulmonary edema | renal failure
elevated lead level on capillary testing must be confirmed by ⬜
venous lead level
_________________
lead tox ➜ neurobehavioral impairment. tx = AVOID LEAD EXPOSURE
Multiple Myeloma MOD
abnml proliferation of plasma cells that produce useless immunoglobulin (IgG , IgA) –> [Osteoclast Activating Factor] which → CUBP
When MM produces IgM = WaldenstroM Macroglobulinemia
CUBP
1.{[CRAB end organ ❌](hyperCalcemia), Renal Failure 2/2 bence jones proteinuria, Anemia_normocytic, Back pain i\lytic bone fx }
_________________
2.[Urine IgG/IgA]get urine immunoelectrophoresis
_________________
3.⚠️ [Bone marrow >10% plasmacytosis]CONFIRMS MM DX
_________________
4.[Protein(serum: M protein, IgG, IgA [monoclonal “M” spike if MGUS & smear: Rouleaux )]
Multiple Myeloma dx - 4
CUBP
1.{[CRAB end organ ❌](hyperCalcemia), Renal Failure 2/2 bence jones proteinuria, Anemia_normocytic, Back pain i\lytic bone fx }
_________________
2.[Urine IgG/IgA]get urine immunoelectrophoresis
_________________
3.⚠️ [Bone marrow >10% plasmacytosis]CONFIRMS MM DX
_________________
4.[Protein(serum: M protein, IgG, IgA [monoclonal “M” spike if MGUS & smear: Rouleaux )]
Spinal Cord Compression can be from DJD, Epidural Abscess or Tumor
Which Cancer metastasis are associated with Tumor Spinal Cord Compression? - 5
- Prostate
- Renal
- Lung
- Breast
- Multiple Myeloma
a. acute TOXIC ingestion of ASA = taking [__________mg/kg in 1 dose].
_________________
B. TOXIC ASA ingestion will lead to what 3 acid-base disturbances?
a. >100
_________________
b.
1st: ASA-induced[AG metabolic acidosis] ➜
2nd:compensatory[respiratory alkalosis] =
3rd: [MIXED AG metabolic acidosis WITH respiratory alkalosis]
“Air HOT/[Sound ringing-stomach sick]/Air fast”
quick way to distinguish acute Aspirin OD from acute Acetaminophen OD?
Acute Aspirin ingestion ➜ vomiting
“acute A S A gets you Sick to ur Stomach(vomiting)!”
classic triad for Aspirin OD
ASA –> [Mixed Respiratory alkalosis + AG metabolic acidosis] (Normal pH/DEC PCO2/DEC HCO3)
- [Air is Fast (Tachypnea)]
- {[Sound ringing (Tinnitus)] … and [Stomach Sick (Vomiting)]}
- [Air is Hot (Fever]
Gold standard for TIA prophylaxis ?
How does it work?
ASA;
COX inhibitor –> DEC [Thromboxane A2] –> DEC Platelet aggregation and vasoconstriction
✏️Activated platelet → secretes fibrinogen and {[ThromboxaneA2 & ADP] → [INC (Gp2b/3a_platelet fibrinogen R) expression on platelets]}
Explain Winter’s Formula -4
⏸️[COMPENSATED arterial PaCO2(compensating for acute metabolic∆|acute renal∆)]
⏸️should be within
⏯️ {+/- 2 of[1.5 x HCO3 + 8]} …
⏏️…If not = [mixed acid/base picture]
What lab abnormalities indicate Hemodialysis to treat Aspirin OD -3
- [initial salicylate > 160] mg/dL
- [6 hr salicylate > 130]
- persistent acidosis pH < 7.1
DVT/PE are treated with [rafX Factor 10a inhibitor] or [Warfarin]
Compare the following parameters between
[rafX Factor 10a inhibitor] and [Warfarin]
__________________
a. Mechanism of Action
b. Therapeutic onset
c. Overlap needed?
D. Laboratory monitoring
DVT #1 = {[rafX Factor 10a inhibitor] x ≥3mo}tx
T or F
________________
“[Antiplatelets (like ASA, CLOpidogrel, ABCiximab)] are NOT used in DVT tx”
TRUE
T or F
“Patients who develop [HITT2 or HITT1] can use Heparin after 6 months”
FALSE!!!!!!!!
PATIENTS WITH HITT ARE HEPARIN-BANNED FOR LIFE!!
Name the [Direct Thrombin Factor 2a inhibitors] (3)
DAB
1. Dabigatran
2. Argatroban
3. Bivalirudin
-bivalirudin is related to hirudin (anticoagulant leeches use)
-Use [Direct Thrombin Factor 2a inhibitors] in patients with HIT
mgmt for HITT2 -3
________________
HITT2 = Heparin Induced Thrombocytopenia type 2
- DISCONTINUE ANY HEPARIN
- start DAB[Direct Thrombin Factor 2a inhibitors] or
- start [fondaparinuX(factor 10a inhibitor)]
How do you calculate liklihood of [HITT2 (Heparin Induced Thrombocytopenia type 2)]? -4
HITT2 = all 4T score
- Thrombocyte DEC GOE30-50%
- Timing onset 5-10d after Heparin exposure (or 1d if previous exposure)
- Thrombosis present
- Took Out other thrombocytopenia causes
[Heparin Induced Thrombocytopenia type1 (HITT1)]
________________
mechanism of disease
[HITT1] = NONIMMUNE Heparin-Platelet clumping] ➜ [platelet > 100K] and resolves spontaneously
“oozing from mucosal sites” is associated with which:
HITT or DIC?
DIC
[Heparin Induced Thrombocytopenia Type2 (HITT2)]
________
mechanism of disease -3
Leukocoria = _____ associated with what kind of Cancer?
WHITE EYE REFLEX;
Retinoblastoma
CP of Acute Intermittent Porphyria - 3
A-I-P causes N-A-P
- Psychosis acute onset
- Abd pain acute onset
- Neuropathy acute onset
Fam hx of this is VERY suggestive of AIP
In Order, List the 5 Enzymes involved in Heme Synthesis
AAPUF
1st: <ALAS | Sideroblastic>
2nd: <ALAD | lead tox>
3rd: <PorphoDeam | AIP>
4th: <UROPorphoDeCARB | PCT>
5th: <Ferrocheletase | lead tox>
________________
enzyme❌is associated with [| ⬜]
isolated ELEVATED IRON is specific to what type of anemia?
Sideroblastic anemia
Anemia with normal iron studies is specific for what type of anemia?
Thalassemia (except 3 gene deletion alpha thalassemia)
Dx = Hgb electrophoresis with genetic studies if alpha thalassemia
Tx for sideroblastic anemia
Pyridoxine B6
Causes of Vitamin B12 deficiency - 6
- PERNICIOUS ANEMIA = MOST COMMON CAUSE
- Vegan/Vegetarian
- Blind loop syndrome (Gastrectomy or RYGB)
- Diphyllobothrium latum
- Pancreatic Insufficiency
- Terminal iLeum damage (Crohns)
how do you differentiate Vitamin B12 deficiency from Folate deficiency
Vitamin B12 isomerizes methymalonyl coA in the spinal cord myelin —> succinyl coA.
Without it –> suBACute combined degeneration
usually manifest as paresthesia/peripheral neuropathy
Peripheral Smear of pt with Sickle Cell Disease
Explain this abnormal finding
Howell Jolly Bodies = [RBC basophilic nuclear remnants(usually removed by spleen macrophages)] = Pts with autosplenectomy/asplenia(i.e. from Sickle Cell Disease) = will not be able to remove [Howell Jolly Bodies]
What disease do you see Morulae on peripheral blood smear?
Ehrlichia infection
Do NOT confuse Cryoglobulins with Cold IgM hemolysis
What are [Cryoglobulins type 2] associated with? - 3
- Hep C
- Joint Pain
- Glomerulonephritis
HUS and TTP etx
TTP etx: [A13vM❌(or A13vM inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → severe thrombocytopenia + [RBC shearing ⼀ microangiopathic hemolytic anemia]) → FMNRT TTP sx ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp.
(TTP Tx = EXCHANGE plasma containing anti-A13vM or deficiency of A13vM for normal plasma)
TTP is associated with what conditions? - 5
- CLOpidogrel
- tiCLOpidine
- cyCLOsporine
- AIDS “CLOset gay”
- {PregnancyTTP can onset anytime during [gestation or postpartum pregnancy]} “CLOset Mom”
Unlike HUS, [TTP = (neuro sx (confusion/seizure)) / fever / (plasma EXCHANGE tx)]
TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → FMNRT TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ (TTP Tx = EXCHANGE plasma containing anti-A13vM or deficiency of A13vM for normal plasma)
Tx for Paroxysmal Nocturnal Hemoglobinuria -3
- Prednisone
- Bone Marrow Transplant = cure
- Eculizumab (inactivates C5 complement)
“Pt has intense pruritus after a warm shower”
What is the Diagnosis?
________________
How do you diagnose this?
Polycythemia Vera
________________
JAK2 mutation (r/o hypoxia)
________________
Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more
You must exlude Hypoxia as a cause of ⬆︎RBC
Tx for Polycythemia Vera - 3
- phlebotomy
- hydroxyureaInhibits [Ribonucleotide Reductase] –> inhibits [DNA thymine] synthesis→ ⬇︎cell synthesis
- . [ASA for erythromelalgia (painful red hands from ET or PCV)]
When do you treat Essential Thrombocytosis?-2
ONLY when
- pt>60 yo with sx
OR
- pt>60 with [platelets>1.5million]
Tx for Essential Thrombocytosis - 3
- HYDROXYUREAInhibits [Ribonucleotide Reductase] –> inhibits [DNA thymine] synthesis
- [Anagrelide when RBC is suppressed from Hydroxyurea]
- [ASA for erythromelalgia (painful red hands from ET or PCV)]
DDx for pt presenting with pancytopenia - 7
- ALL
- AML
- [aPL - acute Promyelocytic Leukemia M3 (chromo T15/17)]
- Aplastic Anemia(Radiation|drugs|Viruses|Fanconi|Idiopathic)
- [CIPM - Myelofibrosis (dry tap and tear drop cells)]
- [Myelodysplastic Syndrome (hypercellular bone marrow with ringed sideroblast RBC with Prussian blue )]
- [Hairy Cell Leukemia (dry tap with hypercell bone marrow)]
Acute Leukemia will present with signs of ⬜
________________
Which acute leukemia is associated with [ATRA-all trans retinoic acid]?
PANcytopenia
________________
[aPL - acute Promyelocytic Leukemia M3 (chromo t1517)]
Dx = smear showing blast –> flow cytometry for confirmation
How is the [LAP-Leukocyte Alkaline Phosphatase] test used for Heme/Onc diagnostics? (5)
❗️[CML(9/22 BCRABL)] ⇪ Granulocyte proliferation ⼀
❗️[infection (Leukamoid Stress Reaction)] also ⇪ Granulocyte proliferation ⼀
❗️but [CML Leukemia CA cells] do NOT have high levels of [Leukocyte Alkaline Phosphatase] and
❗️ ❗️ so… ⇪Granulocytes? Check LAP
⭐[CML (LAP elevated?)INFECTION]
_________________
CML: Chronic Myelogenous Leukemia
What are the initial therapies for Chronic Myelogenous Leukemia?-3
________________
What is the ultimate cure?
etx: chromo 922 = BCR ABL gene
tx = [tyrosine kinase inhibitors ( x-tinib)] such as ..
- imatinib
- dasatinib
- nilotinib
Cure = Bone marrow transplant (NEVER the first therapy though)
What disorder does the [Pseudo⼀Pelger Huet anomaly (described as ⬜)] belong to?
✏️[neutrophils with 2-lobe nuclei(instead of the normal 3-lobe nuclei)]
2/2 [de novo mutations vs exposure(radiation, benzene, chemotherapy)] → dysfunctional hematopoiesis (defective cell maturation) of Myeloid cells(risk of transformation to AML :-( )
✏️MyeloDysplastic Syndrome
Describe peripheral blood smear for [(CLL-SLL)-Chronic Lymphocytic Leukemia - Small Lymphocytic Lymphoma]
proliferation of normal and mature (but dysfunctional) B lymphocytes with smudge cells
What is the Richter phenomenon
conversion of CLL–> [high grade lymphoma (DLBL or Prolymphocytic Lymphoma)] which happens in 5% of patients
________________
CLL = mature lymphocytes and smudge cells
Hairy Cell Leukemia Tx?
B cells with filamentous projections on smear
[2CDA_Cladribineadenosine analog]
NonHodgkin Lymphoma Dx? -2
nHL Dx = [Xbx with CTS ➜ Bbx for tx]
_________________
[EXCISIONAL bx with CT staging]
and
[BoneMarrowbx to determine tx]
________________
nHL dx = HODGKIN LYMPHOMA dx- (except HL also requires [⊕ReedSternberg CD15 & CD30 owl eye cells]) =
HL Dx = i\⊕CD15 and ⊕CD30[Xbx with CTS ➜ Bbx for tx]
Tx for
[NonHodgkin Lymphoma with Bsx] (5)
CHOPX
Tx for
[Hodgkin Lymphoma with B sx] (4)
BHOD
Tx for
[NonHodgkin Lymphoma stage _____]
1
2
3
4
1 = Radiation_local
2 = Radiation_local
3 = CHOPX
4 = CHOPX
Tx for
[Hodgkin Lymphoma stage _____]
1
2
3
4
1 = Radiation_local
2 = Radiation_local
3 = BHOD
4 = BHOD
Describe the Staging for [NonHodgkin Lymphoma] (5)
“Tall Brian _F_orgot _M_r. _B_urkitt’s _D_og”
Describe the Staging for [Hodgkin Lymphoma] (5)
For Hodgkin Lymphoma, what are the determinants for prognosis? (2)
STAGING= MOST IMPORTANT PGN > [(Lymphocyte RICH) = GOOD Pgn]
[(Lymphocyte mixed) and (Lymphocyte poor))]= poor pgn
In Heme/Onc what are the MUGA and nuclear ventriculogram used for?
Determine cardiotoxicity for Adriamycin/Doxorubicin tx used for Hodgkin Lymphoma
What are the toxicities for Cisplatin and Carboplatin? - 2
What are the toxicities for Vincristine?
What are the toxicities for Bleomycin and Bulsulfan?
What are the toxicities for Doxorubicin?
What are the toxicities for CYclophosphamide?
What are the toxicities for 5-FU?
What are the toxicities for 6-MP?
What are the toxicities for MTX?
What are the triggers for DIC - 6
“his DIC SCABS Terribly!”
- Sepsis
- CA
- Abruptio placenta or Amniotic fluid embolus
- Burns
- Snake bites
- Trauma –> tissue factor release
DIC activates primary AND secondary coagulation
Tx for DIC - 3
- [FFP(contains clotting factors but req 2L dose)]
- [Cryoprecipitate(contains clotting factors, vWF, and replaces fibrinogen if FFP doesn’t work)]
- Platelets if < 50K
“his DIC SCABS Terribly!”
Which type of clots are more common with HIT?
________________
dx for HITT2?-2
Venous
________________
[Platelet factor 4 Ab on ELISA] or [Serotonin release assay]
Bernard Soulier cp - 2
- Superficial Bleeding out of proportion to the degree of thrombocytopenia
- GIANT platelets
etx = absent [platelet glycoprotein 1B R] for von willebrand factor to bind to
Fanconi anema is an auto recessive disorder with what etx?
_________________
cp?-3
[Fanconi P-A-N anemia] = DNA repair defect
that → [CPMSC❌ (failure/destruction/suppression)) = Aplastic Anemia] →
- [Aplastic AnemiaPANcytopenia marrow failure] → [mucosal bleeding/petechiae], [NORMOcytic but hypOproliferative anemia], infection
- morphological changes
- growth stunt
🔎CPMSC = [Common Progenitor Myeloid Stem Cell]
🔎P-A-N = [Pancytopenic-Aplastic-Normocytic]
Laboratory results for Chronic Myelogenous Leukemia?-4
- ⬆︎ABSOLUTE BASOPHILIA
- ⬆︎⬆︎⬆︎LEUKOCYTOSIS
- shift tward precursor cells (myelocytes or promyelocytes)
- ⬇︎Leukocyte Alkaline Phosphatase (LAP)
Cure = Bone marrow transplant (NEVER the first therapy though)
etx = 922 BCRABL philadelphia chromosome
Dx for Chronic Myelogenous Leukemia?-3
LOW Leukocyte Alkaline Phosphatase score (marker of neutrophil activity and differentiates from leukomoid rxn)
Factor 5 Leiden MOD
_________________
how does this affect aPTT and PT/INR
AUTO DOM point mutation in Factor 5 gene –> RESISTANCE TO PROTEIN C (which is supposed to inactivate Factor 5). This –> Hypercoagulability
aPTT AND PT/INR may both be normal!
Dx for Hereditary Spherocytosis - 3
E5 with Acid
[Eosin 5 maleimide binding flow cytometry] WITH [Acidified glycerol lysis test]
OR
Osmotic fragility test but it has low sensitvity
Lab findings = ⬆︎Mean Corpuscular Hgb Concentration
Hereditary Spherocytosis MOD -3
[🩸 = “RBC membrane”]*
🟠[🩸Spectrin’s inability to anchor [AUTO DOM defective 🩸Ankyrin] → [🩸 bleb formation] –> splenic macrophages remove but hemolyze/damage RBC in process➜ [JAUNDICE unconjugated hyperbilirubinemia],
_________________
🟠 over time, splenic macrophages hypertrophy → [SPLENOMEGALY]. And [RBC∆(⬇︎MCV with ⇪MCHC, biconcave → spherical) → [High RDW] = [“SPHEROCYTE” RBCs]
_________________
🟠 ULTIMATELY, [SPHEROCYTE RBC ∆ (small, spherical, no central pallor)] → [splenic macrophage premature removal altogether → [*eXtravascular hemolytic anemia ] Tx = Splenectomy
🔎MCHC = Mean Cell Hgb Concentration
Triad = Jaundice, Splenomegaly, Hemolytic Anemia
Which hematological abnormality is Acute Cholecysitis a major complication of?
{[Hereditary SpherocytosisChronic Hemolysis]
Triad = Jaundice, Splenomegaly, Hemolytic Anemia
HS –(pigmented bilirubin gallstones)–>[Acute Cholecystitiss]}
Although it is a procoagulant, why is lupus anticoagulant called an anticoagulant?
because ONLY in the petri dish, it causes prolonged aPTT
A white male presents with megaloblastic anemia, atrophic glossitis, vitiligo and neuro problems…
all consistent with Vitamin B12 deficiency
What is likely the cause?
GENETIC! Whites of Northern European ancestry naturally develop Pernicious Anemia
also, Pernicious Anemia ⬆︎ risk for gastric ADC
Describe etx for Warfarin induced skin necrosis (4)
▶Warfarin inhibits epoxide reductase (which reduces⼀activates Vitamin K(K is responsible for maturing [2, 7, 9, 10, Protein C and S]) → ⬇︎[2,7,9,10, Protein C and S]
▶2,7,9,10 = ⬆︎Clotting
▶[Protein C and S] = anti-Clot = Bleeding.
⭐But since Protein C and S are the first to be affected by warfarin, allowing 2,7,9,10 to roam freely and ⬆︎Clotting
Pernicious Anemia is the most common cause of Vitamin B12 deficiency
Pernicious Anemia ⬆︎ risk for developing what type of cancer?
Gastric ADC
What disease should you suspect in a pt with Macrocytic anemia and congenital anomalies?
Diamond Blackfan Syndrome (DBS)
intrinsic defect in erythroid progenitor cells –> ⬆︎apoptosis
Type of Cell? ; Diagnosis?
Atypical Reactive CD8 T cells; Infectious Mononucleosis
MOD for Hairy cell leukemia? ; How is diagnosis made?
B cell neoplasm that infiltrates bone marrow, spleen and peripheral blood; Bone Marrow Biopsy
Diagnosis? ; MOD of this disease?
Gaucher ; lysosomal storage disease
Describe what Total Iron Binding Capacity (TIBC) measures?
“T ransferrin _I_n _B_lood _C_alculation”
-TIBC gives a snapshot (for that that moment in time) of how much iron the body:
-can carry (TIBC indicates total capacity [for that moment] the body has to transport iron). ⇪ capacity/TIBC may indicate ⇪ demand)
-wants to carry (demand)
Describe the following values for Thalassemia:
MCV
Iron
Transferrin Saturation
TIBC
Ferritin
Describe the following values for Sideroblastic Anemia:
MCV
Iron
⬇︎MCV
⬆︎Iron
How do you diagnose [CLLSLL]? (2)
_________________
[CLLSLL-Chronic Lymphocytic Leukemia-Small Lymphocytic Lymphoma]
1.peripheral blood FLOW CYTOMETRYproliferation of [normal, mature (but dysfunctional) B lymphocytes]
with
2.peripheral blood smearsmudge cells
* * *
Suspect CLLSLL in any elderly with dramatic leukocytosis primarily made of lymphocytes
Pt has intense pruritus after a warm shower. Polycythemia Vera is diagnosed
What is the difference between Phlebotomy and Plasma exchange?
Phlebotomy (tx for PV) removes cells while Plasma exchange only removes substances (Antibodies, immune complexes, toxins)
Identify cells ; What 2 diseases are they associated with?
“Discern {[EBR[Echinocyte/Burr=Renal🌀] from ASL[Acanthocyte/Spur(ky)=Liver🌀]] ⼀red blood cells}!”
_________________
[Acanthocyte Spurky RBC] = [Liver🌀 | Cholesterol❌]
[Acanthocyte Spur RBC] are Spurky = Spiky!
🌀 = disease
What blood disorder should be suspected in a pt with ⬆︎Mean Corpuscular Hgb Concentration?
Hereditary Spherocytosis
Tx for iron deficiency anemia?
[ferrous sulfate 2+ _ heme iron]
”give *us 2 more iron”*
“Pt has intense pruritus after a warm shower”
What is the Dx? ; Why does this happen?
Polycythemia Vera
________________
Heat ⬆︎Basophils (rare but can become AML) –> ⬆︎Histamine release
________________
Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more
Hairy Cell Leukemia dx?-2
B cells with filamentous projections on smear
[TRAP-Tartrate Resistant Acid Phosphatase]
or
CD11c
NonHodgkin Lymphoma cp - 2
- painLESS LAD
- B sx (Fever, Night sweats, Wt Loss)
Dx = EXCISIONAL bx with staging via CT and BMbx to determine tx
THIS IS THE SAME AS HODGKIN LYMPHOMA - except HD has ReedSternberg owl cells
[Acute Intermittent Porphyria] dx
________________
What factor of a pts hx suggest [Acute Intermittent Porphyria] ?
[(⬆︎Porphobilinogen) in Urine]
________________
Fam hx of similar sx
Name the substrate for the [heme synthesis enzyme]
<UROPorph | PCT>
(UROPorphyrinogen)
⬇︎
<UROPorphoDeCarb | PCT> “UPDC PCT”
__________________
AAPUF
enzyme is associated with [| x]
Name the substrates for the [final heme synthesis enzyme]
<Ferrochelatase | lead tox> - 3
{[CoproPorphyrinogenin cytoplasm (enters mitochondria)→ Protoporphyrinin mitochondria] + Fe2+ }
⬇︎
<Ferrochelatase | lead tox>
⬇︎
⭐HEME⭐
__________________
AAPUF
enzyme is associated with [| x]
Name the substrate for the [heme synthesis enzyme]
<ALAD | lead tox>
(ALA)
⬇︎
<ALAD | lead tox>
__________________
AAPUF
enzyme is associated with [| x]
Name the substrate for the [heme synthesis enzyme]
<Porph | AIP>
(Porphobilinogen)
⬇︎
<Porph | AIP>
__________________
AAPUF
enzyme is associated with [| x]
Name the substrates for the [heme synthesis enzyme]
<ALAS | Sideroblastic> - 3
[(Glycine) + (Succinyl CoA) + (Pyridoxine B6)]
⬇︎
<ALAS | Sideroblastic>
__________________
AAPUF
enzyme is associated with [| x]
What is the differnece between Exertional heat stroke and NonExertional heat stroke?
_________________
cp for heat stroke (3)
cp = HOT
[Head ❌(CNS dysfxn/confusion/weakness/seizures)]
[Organ ❌(ARDS/Pulm Edema/NV/Liver Injury/AKI/Rhabdomyolysis/DIC)]
[Temp core > 40C (104F)]
heat stroke management
cp = HOT
tx = evaporative /convective cooling with water misters and fan
Licorice is commonly found in ⬜
________________
how does it affect BP?
herbal teas;
[Anemia = ⬜]
What are the 3 Categories of Anemia?
⬇︎O2 carrying capacity in blood
CLDP
LDP
[Loss of RBC Blood Volume](Melena, Hematochezia, Ulcers, Fibroids)
[Destruction of RBC (Hemolytic Anemia)]Intrinsic|Extrinsic in Intravascular|Extravascular
[Poor production of RBC (⬇︎Erythropoiesis)]
definition of
Anisocytosis
varying sizes of RBC
definition of
Poikilocytosis
varying shaPes of RBC
(image) is activated by ⬜ and forms ⬜
[Thrombocyte platelet]
_________________
[endothelial tissue injury in 1º hemostasis] ;
[Thrombocyte platelet] plug after aggregating with other [Thrombocyte platelets] and interacting with fibrinogen
⅓ platelets are stored in spleen
Name all the Leukocytes (Most to Least abundant) (5)
Neutrophils Like Making Everything Better
Neutrophils
Lymphocytes
Monocyte_macrophages
Eosinophils
Basophils
Describe the breakdown for hematopoietic neoplasia (6)
start with cell age
Acute
1. [Acute>Lymphoid]= ALL
2. [Acute >Myeloid]= AML
* * *
Chronic
- [Chronic>Lymphoid>Chronic Leukemia= C|H|A]
- [Chronic>Lymphoid>Lymphoma={Hodgkin vs [NonHodgkin”🆃all 🅱rian <span>F</span>orgot <span>M</span>r <span>B</span>urkitt’s <span>D</span>ogg” | ]}
- [Chronic>Lymphoid>Plasma cell disorders=MultipleMyeloma | Waldenstrommacrogloulinemia, MGUS]
_________________
- [Chronic>Myeloid>Myeloproliferative disorders* (Constitutively Activated Tyrosine Kinase → Myeloproliferative disorder) = 1|P|E|C]([1º Myelofibrosis/CIM] | [Polycythemia Vera] | [Essential Thrombocytosis] | [Chronic Myelogenous Leukemia]
[⇪ band cellsAKA “bandemia” (which are defined as ⬜)] indicates what? (2)
immature neutrophils;
{ [⇪ mYeloblast (precursor to BENgranulocytes)] proliferation( = bacterial infections or CML)]}
[Burkitt Lymphoma - EBV] is associated with ___ translocation and overexpression of ____ and what 2 symptom forms?
Describe the Histo (4)
[8cMyc ➜ 14IG-Heavy Chain]; [cMYC (and EBV INC B- cell proliferation–>INC translocation risk)] = [Endemic_Africa (latent infection w/EBV) → Mandibular lesion] vs [Sporadic_USA → abd/pelvis lesion]
Histo =
- “Starry Sky appearance”
- [Diffuse mid-sized lymphocytes with interspersed macrophages (arrows)
- basophilic cytoplasm
- and high [Ki-67 fraction-proliferation index]
Describe
Mast cell (3)
- binds Fc portion of [IgE⼀antigen complex] → cross-links → degranulation(releases histamine, heparin and eosinophil chemotaxis factors)
- mediates allergic reaction / [Type 1 hypersensitivity]
- [Cromolyn sodium (asthma px)] prevents mast cell degranulation
Sickle cell anemia is ___(mode of inheritance) and should be diagnosed with what?
auto recessive; [HgB electrophoresis( will show HgbS with NO_HgbA)] determines carrier status
[GlutaMATE –> Valine @ 6th position of the [β-chain globin]
explain how [Vitamin K] works (3)
a. enteric bacteria synthesizes [oxidized_inactive vitamin K] ( this means neonates will not have Vitamin K and need px!)
b. [oxidized_inactive vitamin K] is [reduced⼀activated] by [epoxide reductase (inhibited by Warfarin)] → [reduced_ACTIVATED vitamin K]
c. [_reduced__ACTIVATED vitamin K] acts as cofactor for maturing factors [2,7,9,10,C,S] during synthesis which → [(2,7,9,10)Pro_2ºCoagulation] and [(Protein C,S)Anticoagulation(EARLY Warfarin☠️)]
—————
☠️: [(Protein C,S)Anticoagulation* are *(EARLY Warfarin Victim☠️)] ➜ 1st to ⬇️ when Warfarin inhibits epoxide reductase = IMPORTANT SINCE ALLOWS FOR 1 WEEK/TEMPORARY UNINHIBITED → [(2,7,9,10)Pro_2ºCoagulation] -➜ = [Warfarin induced skin necrosis]
[Protein C and S] are involved with ([⬜ anti | pro]coagulation)
How do they work?
anti
_________________
1-{[endothelial cell thrombin-thrombomodulin complex]} activates [Protein C]
2-[activated Protein C] uses [Protein S] to [cleave ( = inactivate)] 5a and 8a of coagulation cascade
What is Ristocetin?
During [Primary hemostasis platelet plugging] vs lab diagnostics,
Ristocetin activates [vWF (which is already bound to subendothelial collagen exposed from injury)] to bind to [Gp1b_platelet aDherence R]
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (2)
“Discern {[EBR[Echinocyte/Burr=Renal🌀] from ASL[Acanthocyte/Spur(ky)=Liver🌀]] ⼀red blood cells}!”
_________________
[Acanthocyte Spurky RBC] =
1. [Liver🌀 :Liver Disease] or
2. [Cholesterol❌:abetaLipoproteinemia cholesterol dysregulation]
[Acanthocyte Spur RBC] are Spurky = Spiky!
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (4)
[Schistocyte helmet RBC]
_________________
- DIC
- TTP
- HUS
- [traumatic hemolysis(i.e. mechanical heart valve )]
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (3)
[Spherocyte RBC]
_________________
- Hereditary Spherocytosis
- G6PD deficiency
- autoimmune hemolysis
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (2)
[Macro-ovalocyte (macrocyte) RBC]
_________________
- Megaloblastic anemia (am_FOB)
- Nonmegaloblastic anemia(an_LAR)
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology?
[Ringed sideroblast in RBC]
_________________
Sideroblastic anemia (pathologic excess iron in mitochondria)
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology?
[Teardrop RBC]
_________________
1º Myelofibrosis
(bone marrow infiltration → forces RBC out of bone marrow → tear shape)
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology?
[Bite RBC]
_________________
G6PD deficiency
(RBC oxidation of hgb sulfhydryl groups < 2/2 loss of G6PD buffer system > → [denatured hgb precipitation (AKA Heinz bodies)]. [Spleen macrophage Phagocytic damage] of these [Heinz bodies] →RBC membrane damage → [Bite RBC]
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology?
[howell Jolly bodies in RBC]
_________________
Spleen❌ (functional hypOsplenia/asplenia)
(basophilic nuclear remnants in RBC that are usually removed by spleen macrophages)
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology?
[Heinz bodies in RBC]
_________________
G6PD deficiency
(oxidation of hgb sulfhydryl groups → [denatured hgb precipitation (AKA Heinz bodies)]. [Splenic macrophage Phagocytosis] of these [Heinz bodies] →RBC membrane damage → [Bite RBC](and after so many bites → unstable Spherocyte RBC))
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (4)
[Basophilic stippling of RBC]
_________________
“_Basi_cally, ACid alcohol is Le-Thal
- Anemia of Chronic Disease
- alcohol abuse
- Lead poisoning
- Thalassemia
Identify finding (in image) from pt’s peripheral blood smear
_________________
What is the associated pathology? (4)
[Target RBC]
_________________
“HALT! said the LIVid hunter to his target”
- HbC
- Asplenia
- LIVER disease
- Thalassemia
What is [Plummer-Vinson syndrome]? (3)
“PVS is IDA as IEA”
[iron deficiency anemia (microcytosis, hypOchromia)] that manifest as …
⼀Iron deficiency anemia
⼀Esophageal webs
⼀Atrophic glossitis
Describe [Normal Adult hemoglobin (Hgb A)] (3)
[Normal Adult Hgb (Hgb A)] =
[4 globin chains: (α2 β2)] =
globin:[< 2 x α(chromo 16: 2 alleles per 1 globin) > + < 2 x β(chromo 11: 1 allele per 1 globin) >] =
alleles: [chromo 16<αα / αα > + chromo 11<β / β >]
Sideroblastic Anemia etx (5)
★ → defect in ALAS of heme synthesis →[isolated elevated iron(specifically in mitochondria)] → [Bone Marrow Ringed sideroblast RBC (that contain iron-laden mitochondria)] ➜
★ [⇪ iron / ⇪ FerriTin / nl_TIBC]
★ [X-linked],
★ [acquired (myelodysplastic syndromes)],
★ {reversible (BAIL Out the Copper) with [Pyridoxine B6 (cofactor for ALAS) tx]}
Sickle Cell Disease etx (8)
- [HgbA gene point mutation causes [Glutamic acid → Valine] at [position 6 of the β chain monomer] = HgbS =
- [DICK/DeOxygenation] of HgbS → [CAP-(crystallization aggregative polymerization)] → [HgbSS|SC Sickle Shaped RBC].
- Will Un-sickle once O2 is bound again but eventually → RBC Membrane stiffness →
a. Chronic Hemolytic Anemia
- Intravascular hemolysis 2/2 membrane instability
- Extravascular hemolysis 2/2 red pulp splenic sequestration of sickles → ultimately causes splenic hypoxia/fibrosis → [auto/functional splenectomy]
b. Small Vessel Occlusion by sickles → Vasoocclusive Pain Crisis
c. Aplastic Crisis (2/2 Parvo B19 ⬇︎ erythroid precursors)
d. [Sickle RBC = ⬇︎1/2 life] → Anemia - HgbF is protective
Which lab is used to follow disease activity in SLE? and why?
*dana *
antidsDNA (indicates and tracks development of lupus nephritis)
ITP
etx (4)
[Immune Thrombocytopenic Purpura]
[anti-platelet Ab(against platelet_Gp2b/3A)] start binding to [Platelet R 2b/3A] = forms [Antibody/Platelet complex] (no obvious cause > precedes viral/HIV?HCV? infxn)
_________________
→ splenic macrophages removes [Ab/Platelet complex] from circulation ➜ [isolated thrombocytopenia <100K]
_________________
- → superficial/mucosal bleeding
- [⇪ megakaryocytes on bone marrow biopsy]
_________________
-NORMAL SPLEEN
-normal clotting factors
-normal clotting PT/aPTT
Strongly associated with HIV and HCV
