✅Hematology/Oncology Flashcards
1
Q
Dx test for Iron Deficiency Anemia
________________
why
A
FerriTin < 15
________________
measures iron storage and is specifically for IDA
Iron,TIBC,Transferrin can all be low in Anemia of Chronic Dz as well
2
Q
Causes of Normocytic Anemia
NON-Hemolytic (normal or low Retic count) -5
A
3
Q
Causes of Normocytic Anemia
Hemolytic (INC Retic count) - 10
A
4
Q
Is Haptoglobin ⬆︎ or ⬇︎ in Hemolytic Anemia?
________________
Why?
A
DECREASED
________________
Liberated Hgb (after RBC hemolysis) BINDS to serum Haptoglobin –> HgbHaptoglobin complex –> Cleared by Liver
Haptoglobin picks up Haphazard hgb
5
Q
Lab markers for Hemolytic Anemia -5
A
- ⬇︎Haptoglobin
- ⬆︎LDH
- ⬆︎UnConjugated bilirubin
- Hgbnuria (Reddish brown urine)
- DAT (Direct Antiglobulin Coombs Test) -detects antibody-mediated hemolysis
6
Q
3 main methods of developing iron deficiency
A
- ⬇︎ Intake (PO vs GI absorption)
- ⬆︎Output (menorrhagia/hematuria/hemorrhage)
- ⬆︎ Requirement (pregnancy)
7
Q
High RDW typically indicates what etx -4
A
- Mixed deficiency
- Recent Hemorrhage
- Iron deficiency Anemia (late - microcytic)
- Vit B12/Folate deficiency (Macrocytic)
8
Q
What about FerriTin makes it non-guaranteed lab value for iron changes?
A
FerriTin is also an acute phase reactant and ⬆︎ with any stress
9
Q
Spinal Cord Compression can be from DJD, Epidural Abscess or Tumor
Which Cancer metastasis are associated with Tumor Spinal Cord Compression? - 5
A
- Prostate
- Renal
- Lung
- Breast
- Multiple Myeloma
10
Q
Which has a longer onset of action: Ibuprofen or Naproxen?
A
Naproxen
11
Q
T or F: Brain Metastasis from NonSOLC is Chemosensitive
A
FALSE!
NonSOLC is NONSensitive
12
Q
CP of Acute Intermittent Porphyria - 3
A
AIP causes PAN
- Psychosis acute onset
- Abd pain acute onset
- Neuropathy acute onset
Fam hx of this is VERY suggestive of AIP
13
Q
[Acute Intermittent Porphyria] dx
________________
What factor of a pts hx suggest [Acute Intermittent Porphyria] ?
A
[(⬆︎Porphobilinogen) in Urine]
________________
Fam hx of similar sx
14
Q
Name the substrates for the [heme synthesis enzyme]
<ALAS | Sideroblastic> - 3
A
[(Glycine) + (Succinyl CoA) + (Pyridoxine B6)]
⬇︎
<ALAS | Sideroblastic>
__________________
AAPUF
enzyme is associated with [| x]
15
Q
Name the substrates for the [final heme synthesis enzyme]
<Ferrochelatase | lead tox> - 3
A
[(CoproPorphobilinogen) ➜ (((Protoporphyrin + Fe2+ )))]
⬇︎
<Ferrochelatase | lead tox>
__________________
- AAPUF*
- enzyme is associated with [| x]*
16
Q
Name the substrate for the [heme synthesis enzyme]
<ALAD | lead tox>
A
(ALA)
⬇︎
<ALAD | lead tox>
__________________
- AAPUF*
- enzyme is associated with [| x]*
17
Q
Name the substrate for the [heme synthesis enzyme]
<PorphoDeam | AIP>
A
(Porphobilinogen)
⬇︎
<PorphoDeam | AIP>
__________________
- AAPUF*
- enzyme is associated with [| x]*
18
Q
Name the substrate for the [heme synthesis enzyme]
<UROPorphoDeam | PCT>
A
(UROPorphobilinogen)
⬇︎
<UROPorphoDeam | PCT>
__________________
- AAPUF*
- enzyme is associated with [| x]*
19
Q
In Order, List the 5 Enzymes involved in Heme Synthesis
A
- AAPUF*
1st: <ALAS | Sideroblastic>
2nd: <ALAD | lead tox>
3rd: <PorphoDeam | AIP>
4th: <UROPorphoDeam | PCT>
5th: <Ferrocheletase | lead tox>
________________
enzyme is associated with [| x]
20
Q
When is Cryoprecipitate used?
A
ALTERNATIVE tx to replacing fibrinogen and clotting factors in DIC
this is never used first
21
Q
low Ferritin is specific for what type of anemia
A
iron deficiency anemia
Remember that FerriTin is an acute phase reactant
22
Q
What does TIBC measure
A
unbound iron sites on transferrin
i.e. will be elevated in IDA but low in Anemia of Chronic Disease
23
Q
isolated ELEVATED IRON is specific to what type of anemia?
A
Sideroblastic anemia
24
Q
Anemia with normal iron studies is specific for what type of anemia?
A
Thalassemia (except 3 gene deletion alpha thalassemia)
Dx = Hgb electrophoresis with genetic studies if alpha thalassemia
25
Tx for iron deficiency anemia?
ferro**us** sulfate 2+
26
Tx for sideroblastic anemia
Pyridoxine B6
27
Causes of Vitamin B12 deficiency - 6
1. **PERNICIOUS ANEMIA = MOST COMMON CAUSE**
2. Vegan/Vegetarian
3. Blind loop syndrome (Gastrectomy or RYGB)
4. Diphyllobothrium latum
5. Pancreatic Insufficiency
6. Terminal iLeum damage (Crohns)
28
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 peripheral neuropathy*
29
Causes of Folate deficiency - 4
1. Goat Milk diet (has no folate)
2. Psoriasis
3. Phenytoin
4. Sulfa drugs
30
Chronic hemolysis is associated with what type of gallstones?
**Pigmented** bilirubin gallstones
31
In sickle cell, what causes vasoocclusive crisis -4
**DICK**
1. **D**ehydration
2. **I**nfection
3. **C**old temp
4. **K**ant breathe (HYPOXIA)
32
Sickle Cell Disease dx -2
Initially: Peripheral Smear (since SCtrait won't have sickled cells)
## Footnote
**Confirmation: Hgb Electrophoresis**
33
What type of figures are found on smear in Sickle Cell Disease
Howell Jolly Bodies (left over nuclear material in pts who don't have a spleen to remove it)
34
What disease do you see [Bite keratocyte] cells?
G6PD deficiency
35
What disease do you see Morulae on peripheral blood smear?
Ehrlichia infection
36
How does ParvoB19 affect Sickle Cell Disease pts
parvoB19 causes Aplastic Crisis which freezes any further reticulocyte development from marrow. SC pts usually always have high reticuloctye counts but when hit with parvoB19, they suddenly have a drop
37
MOD for Warm IgG hemolysis
Autoimmune Ab **in the spleen or liver** bind to RBC and remove small amounts of the membrane --\> microspherocytes
38
Tx for [Warm IgG Hemolysis] - 4
1st = [**\*\* CTS initially \*\*** --\> IVIG]
2nd = [Splenectomy ---\> Rituximab]
39
What are the triggers of [Cold IgM hemolysis] - 3
**cold** weather is **M****MM**iserable
1. **M**ononucleosis EBV
2. **M**ycoplasma PNA
3. **M**acroglobulinemia Waldenstrom
*occurs in colder (peripheral) parts of body and resolve with warming up body*
40
Which [Warm IgG hemolysis] tx can **NOT** be given to [Cold IgM hemolysis] - 2
CTS
SPLENECTOMY
41
Do NOT confuse Cryoglobulins with Cold IgM hemolysis
What are Cryoglobulins associated with? - 3
1. Hep C
2. Joint Pain
3. Glomerulonephritis
42
List the 4 main characteristics of G6PD deficiency
**Stress** makes me eat **bites** of **fava beans** with **Heinz** ketchup
1. oxidant **Stress** on RBC from lack of glutahione reductase --\> hemolysis and SUDDEN BACK PAIN
2. **B****ite** cells
3. Induced by **fava beans**, macrobid, sulfa, primaquine, infection
4. **Heinz** bodies (RBC inclusions seen after crystal violet staining)
*Almost always in Black/Mediterranean Men with sudden Anemia*
43
HUS and TTP etx
TTP WILL HAVE MORE NEURO SX and it's tx = plasma **exchange**
*otherwise, HUS and TTP present the same*
44
TTP is associated with what conditions? - 4
1. Clopidogrel
2. Ticlopidine
3. Cyclosporine
4. AIDS
## Footnote
*Unlike HUS, TTP will have neuro sx (confusion/seizures) in addition to Fever*
45
MOD for Paroxysmal Nocturnal Hemoglobinuria
[CD55/59 Decay Accelerating factor] (responsible for deactivating complement) of the RBC, WBC and platelets is produced less
so...
nocturnal respiratory acidosis (shallow breathing during sleep) --\> complement activation --\> complement binds to RBC, WBC and platelets --\> nocturnal hgbnuria/⬆︎infection/thrombosis
46
What is the most common cause of death in Paroxysmal Nocturnal Hemoglobinuria
Thrombosis
## Footnote
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
*(Mesenteric or Hepatic veins)*
47
Tx for Paroxysmal Nocturnal Hemoglobinuria -3
1. Prednisone
2. Bone Marrow Transplant = cure
3. Eculizumab *(inactivates C5 complement)*
48
Treatment for Aplastic Anemia is Bone Marrow Transplant
At what age does [bone marrow transplant] become unavailable?
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
what are the alternatives then?-2
\>50 yo
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
[AntiThymocyte Globulin and Cyclosporine]
49
"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*
50
"Pt has intense pruritus after a warm shower"
What is the Dx?
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
How do you diagnose this?
Polycythemia Vera
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Dx = JAK2 mutation
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
* Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
* You must exlude Hypoxia as a cause of ⬆︎RBC*
51
Tx for Polycythemia Vera - 2
1. phlebotomy
2. hydroxyurea
52
When do you treat Essential Thrombocytosis?-2
ONLY when
## Footnote
1. pt\>60 yo with sx
OR
2. pt\>60 with [platelets\>1.5million]
53
Tx for Essential Thrombocytosis - 3
1. **HYDROXYUREA**
2. Anagrelide when RBC is suppressed from Hydroxyurea
3. ASA for erythromelagia (painful red hands from ET)
54
Aplastic Anemia and Myelofibrosis both present as Pancytopenias
What are 2 discerning factors?
1. Myelofibrosis occurs in older people
2. Myelo = teardrop shaped cells from cells struggling to get out of **fibrosed bone marrow**
3. Myelo tx = [Thalidomide and Lenalidomide] = bone marrow production stimulators
55
*Aplastic Anemia and Myelofibrosis both present as Pancytopenias*
Tx for Myelofibrosis -2
[Thalidomide and Lenalidomide]
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
*( bone marrow production stimulators)*
56
Acute Leukemia will present with signs of ⬜
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Which acute leukemia is associated with DIC?
**PAN**cytopenia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
acute promyelocytic M3 (chromo 1517)
*Dx = smear showing blast --\> flow cytometry for confirmation*
57
Acute Leukemia will present with signs of \_\_\_\_\_
Which acute leukemia is associated with Auer rods (eosinophilic inclusions)?
**PAN**cytopenia ; promyelocytic M3 (chromo 1517)
## Footnote
*Dx = smear showing blast --\> flow cytometry for confirmation*
58
Acute Leukemia will present with signs of ⬜
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Which acute leukemia is associated with Myeloperoxidase?
**PAN**cytopenia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Myelocytic
*Dx = smear showing blast --\> flow cytometry for confirmation*
59
DDx for pt presenting with pancytopenia - 5
1. Acute Leukemia
2. Aplastic Anemia
3. Myelofibrosis (dry tap and tear drop cells)
4. Myelodysplastic Syndrome (hypercellular bone marrow with ringed sideroblast with Prussian blue )
5. Hairy Cell Leukemia (dry tap with hypercell bone marrow)
60
Acute Leukemia will present with signs of ⬜
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Which acute leukemia is associated with [ATRA-all trans retinoic acid]?
**PAN**cytopenia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
[promyelocytic M3 (chromo 1517)]
*Dx = smear showing blast --\> flow cytometry for confirmation*
61
Which acute leukemia is intrathecal MTX given to prevent relapse?
**ALL** is treated with intrathecal MTX to prevent CNS releapse
62
How is the [LAP-Leukocyte Alkaline Phosphatase] test used for Heme/Onc diagnostics?
Leukemic cells do NOT have high levels of Alkaline Phosphatase so in CML, LAP will be low
if LAP is high = leukemoid rxn (stress rxn)
63
Other than [LAP-Leukocyte Alkaline Phosphatase] test, what else can be used to diagnose CML?
[BCR-ABL 922] PCR
64
Out of the Myeloproliferative disorders, which has greatest potential for transformation into Acute Leukemia BLAST CRISIS?
CML
65
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 ..
1. imatinib
2. dasatinib
3. nilotinib
Cure = Bone marrow transplant (NEVER the first therapy though)
66
In pt presenting with acute leukostasis reaction, what is the FIRST important thing to do for them?
Leukaphresis (establish dx AFTER!)
67
Myelodysplastic Syndrome is a PreLeukemic disorder
Why do most pts never actually develop AML from Myelodysplastic Syndrome?
They die of infection or bleeding FIRST
## Footnote
MDS causes pancytopenia despite hypercellular bone marrow
68
Which chromosomal abnormality is Myelodysplastic Syndrome associated with?
5q deletion (these pts have better pgn)
69
What disorder does Pelger Huet cells belong to?
## Footnote
*Cell with Bilobed nucleus*
MyeloDysplastic Syndrome
70
Describe peripheral blood smear for CLL-Chronic Lymphocytic Leukemia
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
71
What is the Richter phenomenon
conversion of Chronic Lymphocytic Leukemia --\> high grade lymphoma which happens in 5% of patients
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
*CLL = mature lymphocytes and smudge cells*
72
What px should be given in Chronic Lymphocytic Leukemia
PCP px
73
Tx for Chronic Lymphocytic Leukemia - 2
Stage
[0: ⬆︎WBC] and [1: LAD] = no tx
[3: HepatoSplenomegaly], [4: Anemia], [5:Thrombocytopenia] = Fludarabine and Rituximab(if available)
74
Hairy Cell Leukemia dx?-2
## Footnote
*B cells with filamentous projections on smear*
[TRAP-Tartrate Resistant Acid Phosphatase] or CD11c
75
Hairy Cell Leukemia Tx?
## Footnote
*B cells with filamentous projections on smear*
Cladribine
76
Non-Hodgkin Lymphoma and Chronic Lymphocytic Leukemia both involve lymphocyte proliferation
What is the major difference
NHL = solid mass (lymph nodes and spleen)
CLL = Circulating liquid mass (so use flow cytometry of peripheral blood to diagnose)
77
NonHodgkin Lymphoma cp - 2
1. pain**LESS** LAD
2. 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
78
NonHodgkin Lymphoma Dx? -2
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
79
Tx for NonHodgkin Lymphoma with Bsx or ≥ Stage 3 -5
80
Tx for NonHodgkin Lymphoma stage 1A and 2A
81
For Hodgkin Lymphoma, what are the determinants for prognosis?
Lymphocyte Predominant = GOOD Pgn
82
Tx for Hodgkin Lymphoma with Bsx or ≥Stage 3 - 4
83
In Heme/Onc what are the MUGA and nuclear ventriculogram used for?
Determine cardiotoxicity for Adriamycin/Doxorubicin tx used for Hodgkin Lymphoma
84
What are the toxicities for Cisplatin and Carboplatin? - 2
85
What are the toxicities for Vincristine?
86
What are the toxicities for Bleomycin and Bulsulfan?
87
What are the toxicities for Doxorubicin?
88
What are the toxicities for Trastuzumab?
89
What are the toxicities for CYclophosphamide?
90
What are the toxicities for 5-FU?
91
What are the toxicities for 6-MP?
92
What are the toxicities for MTX?
93
ITP cp - 2
1. **isolated** thrombocytopenia +/- megakaryocytes on smear (typically after infection)
2. Normal spleen
Strongly associated with HIV and HepC
94
ITP tx - 5
Strongly associated with HIV and HepC
95
What's the most common inherited bleeding disorder
AUTO DOM Von Willebrand Disease
## Footnote
*worst after using ASA*, *detected via Ristocetin cofactor assay*
96
What type of bleeding is seen with Factor 11 Deficiency
USUALLY NO INCREASED BLEEDING
97
What are the triggers for DIC - 6
his DIC **SCABS T**erribly!
1. **S**epsis
2. **C**A
3. **A**bruptio placenta or Amniotic fluid embolus
4. **B**urns
5. **S**nake bites
6. **T**rauma --\> tissue factor release
*DIC activates primary AND secondary coagulation*
98
Tx for DIC - 3
1. Platelets if \< 50K
2. FFP (clotting factors)
3. Cryoprecipitate (replaces fibrinogen if FFP doesn't work)
99
Which type of clots are more common with HIT?
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
dx for HIT?-2
Venous
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
[Platelet factor 4 Ab on ELISA] or [Serotonin release assay]
100
Lupus Anticoagulant dx
Russel Viper Venom test
101
What is the most common complication for pts with Sickle Cell Trait?
Painless hematuria
## Footnote
*Sickle cell is auto recessive*
102
What prophylaxis regimen should pts who've recently had a splenectomy receive afterward?
PCN PO QD
x 5 years
103
Bernard Soulier cp - 2
1. Superficial Bleeding out of proportion to the degree of thrombocytopenia
2. GIANT platelets
## Footnote
*etx = absent platelet glycoprotein 1B-9-5 Receptor for von willebrand factor*
104
In pts with Factor defects (secondary coagulation dysfunction), the long term effect of blood sitting in joints ➜ Hemophilic arthropathy
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
explain how this leads to joint pain?
iron/hemosiderin desposition --\> synovitis and fibrosis of that joint --\> chronic worsening joint pain and swelling
105
The vast majority of Head and Neck CA (i.e. submandibular uL hard non-tender LAD) are what type of CA?
SQC
106
Why are Bisphosphonates given to CA pts? - 2
stabilizes bony metastatic lesions which
1. prevents hypercalcemia of CA
2. prevents fx
107
What is the most common cause of [Folate B9] deficiency in the U.S.?
EtOH
108
What are the major electrolyte changes in Tumor Lysis Syndrome - 4
cytotoxic chemotherapy makes you **PUKE**
## Footnote
1. ⬆︎ **P**hosphate (binds and ⬇︎Ca+ )
2. ⬆︎ **U**ric acid (px = allopurinol and IVF)
3. ⬆︎ **K**+ ( ➜ cardiac arrhythmias)
4. **E**lectrolyte ∆
109
What's the best tx for CA-related anorexia -2
Megestrol progesterone analogue \>\>\> CTS
## Footnote
*Marijuana is only useful in HIV anorexia*
110
\*\*HIGH YIELD\*\*
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
When is EPO indicated for ESRD pts?
What are the side effects of EPO? - 3
Hgb\<10 (use EPO with hct goal of 35%)
## Footnote
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
1. **HTN**
2. HA
3. Flu-like sx
111
How can you differentiate between Bone Marrow Infiltration and Bone Marrow Aplasia using the spleen?
Splenomegaly only occurs in Bone Marrow Infiltration
112
Describe Splenic Sequestration
complication of Sickle Cell Disease in which a pooling of RBC AND PLATELETS within the spleen --\> splenomegaly, pallor and **thrombocytopenia**
113
Sickle Cell Disease pts are at risk of having Aplastic Crisis
What is the difference between Aplastic Crisis and Aplastic Anemia?
Aplastic Anemia is a/w **PAN**cytopenia and can be congenital (fanconi, drug-induced, autoimmune)
114
Sickle Cell Disease pts are at risk of having Aplastic Crisis
Clinical Presentation of this? -3
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
What's the most common cause of this?
1. SUDDEN drop in Hgb with
2. Reticulocyte \< 1%
3. NO Hepatosplenomegaly
Parvo B19!
115
Fanconi anema is an auto recessive disorder that causes \_\_\_\_(micro/normo/macro) anemia
cp?-3
Fanconi MACROcytic anemia
1. PANcytopenia marrow failure
2. morphological changes
3. growth stunt
116
Laboratory results for Chronic Myelogenous Leukemia?-4
1. ⬆︎***ABSOLUTE* BASOPHILIA**
2. ⬆︎⬆︎⬆︎LEUKOCYTOSIS
3. shift tward precursor cells (myelocytes or promyelocytes)
4. ⬇︎Leukocyte Alkaline Phosphatase (LAP)
Cure = Bone marrow transplant (NEVER the first therapy though)
etx = 922 BCRABL philadelphia chromosome
117
Dx for Chronic Myelogenous Leukemia?-3
**LOW** Leukocyte Alkaline Phosphatase score (marker of neutrophil activity and differentiates from leukomoid rxn)
118
lab values for Acute Lymphoblastic Leukemia - 3
1. **\> 25% Lymphoblast from bone marrow bx (THIS IS HOW YOU DIAGNOSE)**
2. TdT positive (TdT is only expressed by preB and preT lymphocytes)
3. PAS positive
cp: LAD, hepatosplenomegaly, thrombocytopenia
119
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 inactive Factor 5). This --\> Hypercoagulability
aPTT AND PT/INR may both be normal!
120
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
121
why are sickle cell patients (both trait and disease) at ⬆︎for benign nocturia
**HYPOSTHENURIA** ; This is when sickles obstruct the vasa rectae of the inner medulla --\> inability to concentrate urine
Happens in Sickle Cell pts
122
G6PD deficiency MOD
**Stress** makes me eat **bites** of **fava beans** with **Heinz** ketchup
Triggers --\> hemolysis
*Almost always in Black/Mediterranean Men with sudden Anemia*
123
Hereditary Spherocytosis MOD
Defect in RBC membrane (ankyrin, band and spectrin) --\> spleen removing the defective parts lil by lil as RBC past which eventually --\> spherocytes which have High mean cell hgb concentration and RBC distribution width
## Footnote
*Triad = Splenomegaly, Jaundice, Hemolytic Anemia*
124
Which type of clots are Heparin Induced Thrombocytopenia pts at risk for, Venous or Arterial?
BOTH
125
A pt coming in with GI distress secondary to EColi should be worked up for what heme condition
Hemolytic Uremic Syndrome HUS HAT
## Footnote
HUS HAT from EColi O157 H7 shiga toxin
**H**emolytic Anemia
**A**cute renal failure
**T**hrombocytopenia
126
What are the 3 examples of Microangiopathic Hemolytic Anemia
HAT (HemolyticAnemia/AcuteRenalFailure/Thrombocytopenia)
1. DIC
2. HUS
3. TTP (will have more neuro signs)
127
how do anabolic steroids affect hematologic lab values? - 3
1. ⬆︎RBCs
2. Hepatotoxic
3. Dyslipidemia
128
what's the most common cause of anemia in premature infants?
Anemia of Prematurity
## Footnote
etx: ⬇︎EPO, shortened RBC life
129
TTP tx
TTP will have more neuro sx and it's tx = plasma **exchange**
130
DIC tx
Cryoprecipitate
## Footnote
(contains clotting factors, fibrinogen and vWF)
131
Which hematological abnormality is Acute Cholecysitis a major complication of?
Hereditary Spherocytosis (from pigmented gallstones)
## Footnote
*Triad = Splenomegaly, Jaundice, Hemolytic Anemia*
132
Although it is a procoagulant, why is lupus anticoagulant called an anticoagulant?
because **ONLY** **in the petri dish,** it causes prolonged aPTT
133
Describe the peripheral blood smear for Chronic Myeloid Leukemia
leukocytosis **with shift toward precursor cells (promyelocytes, myelocytes)**
Cure = Bone marrow transplant (NEVER the first therapy though)
134
What is Trousseau Syndrome?
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
What does it indicate?
hypercoagulable disorder --\> recurrent migratory superficical thrombophlebitis at unusual sites (arm, chest)
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
Pancreatic Cancer (or sometimes stomach, lung or prostate)
135
When is empiric anticoagulation ok for DVT/PE?
ONLY when pt has sx suggesting PE is present . If only DVT sx are present, confirm with US first before anticaogulation
136
How long does it take a vegan to develop macrocytic anemia secondary to vitamin B12 deficiency
≥4 years
137
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**
## Footnote
*also, Pernicious Anemia ⬆︎ risk for gastric ADC*
138
Describe etx for Warfarin induced skin necrosis
Warfarin ⬇︎2, 7, 9, 10, Protein C and S
27910 = ⬆︎Clotting
[Protein C and S] = anti-Clot = Bleeding. But Protein C and S are the first to be affected by warfarin, allowing 27910 to rome freely and ⬆︎Clotting
139
Pernicious Anemia is the most common cause of Vitamin B12 deficiency
Pernicious Anemia ⬆︎ risk for developing what type of cancer?
Gastric ADC
140
What is the mangement for when a pt develops Heparin Induced Thrombocytopenia (HIT) - 3
1st: **STOP HEPARIN OR ENOXPARIN**
2nd: initiate alternative (dabigatran, fondaparinux)
3rd: initiate Warfarin once platelet is \> 150K
141
An elderly pt has minor trauma and develops ecchymoses
What should be first on the DDx for this particular pt?
Senile Purpura (not heme related)
Age-related ⬇︎of elastic fibers in perivascular connective tissue --\> purpura
142
What disease should you suspect in a pt with Macrocytic anemia and congenital anomalies?
Diamond Blackfan Syndrome (DBS)
## Footnote
*intrinsic defect in erythroid progenitor cells --\> ⬆︎apoptosis*
143
What gene abnormality causes Chronic Myelogenous Leukemia?
chromo 922 which forms BCR ABL gene
## Footnote
Cure = Bone marrow transplant (NEVER the first therapy though)
144
Type of Cell? ; Diagnosis?
Atypical Reactive CD8 T cells; Infectious Mononucleosis
145
MOD for Hairy cell leukemia? ; How is diagnosis made?
B cell neoplasm that infiltrates bone marrow, spleen and peripheral blood; Bone Marrow Biopsy
146
Diagnosis? ; MOD of this disease?
Gaucher ; lysosomal storage disease
147
Describe what Transferrin measures?
The amount of iron being transferred in the blood
148
Describe what Total Iron Binding Capacity (TIBC) measures?
The Capacity of Iron transferrin can actually carry
149
Describe the following values for Iron Deficiency Anemia:
MCV
Iron
Transferrin
TIBC
Ferritin
150
Describe the following values for Thalassemia:
MCV
Iron
Transferrin
TIBC
Ferritin
151
Describe the following values for Anemia of Chronic Disease:
MCV
Iron
Transferrin
TIBC
Ferritin
152
Describe the following values for Sideroblastic Anemia:
MCV
Iron
⬇︎MCV
⬆︎Iron
153
How do you diagnose CLL-Chronic Lymphocytic Leukemia?
## Footnote
*Suspect this in any elderly with dramatic leukocytosis primarily made of lymphocytes*
Flow Cytometry of peripheral blood
## Footnote
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
154
An elderly pt who presents with dramatic leukocytosis primarily made of lymphocytes should always make you suspicious of what disease?
Chronic Lymphocytic Leukemia (CLL SLL)
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
155
MOD for Pica
iron deficiency anemia --\> desire to eat **ice, clay, dirt, paper**
156
A pt is newly diagnosed with Head and Neck Squamous cell carincoma
What is the next best step in their diagnostic process?
PANendoscopy (esophagoscopy, bronchoscopy, laryngoscopy)
157
Pt has intense pruritus after a warm shower
What is the Dx? ; What is the tx for this?
Polycythemia Vera ; Phlebotomy
## Footnote
* You must exlude Hypoxia as a cause of ⬆︎RBC*
* Dx = JAK2 mutation*
* Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
158
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)
159
What is the most common type of testicular sex cord stromal tumor? ; What does it secrete?-2
Leydig ; Testosterone AND Estrogen
## Footnote
*All Solid Testicular Tumor Masses should be treated with Radical Orchiectomy*
160
A pt has been diagnosed with a Solid Testicular Tumor Mass via Ultrasound
Tx? -2
**Radical Orchiectomy** --\> additional chemoradiation/surgery based on cell type
161
Identify cells ; What disease are they associated with?
Spur cell acanthocytes ; Liver disease
162
What blood disorder should be suspected in a pt with ⬆︎Mean Corpuscular Hgb Concentration?
Hereditary Spherocytosis
163
cp for Immune Thrombocytopenia ; tx?-3
isolated thrombocytopenia after an infection (usually in a child) ;
1. usually self limited to 6 months = observation
2. IVIG **if bleeding present** OR
3. CTS **if bleeding present**
164
What type of gallstones are pts with Hereditary Spherocytosis at risk for?
Pigmented Bilirubin Gallstones
in Hereditary Spherotycosis, RBC membrane defect can cause intravascular hemolysis
165
Triad for Osler Weber Rendu syndrome
Osler Weber likes to **EAT**
1. **E**pistaxis recurrently
2. **A**V malformations
3. **T**elancietasia
166
Which 2 Vitamins are used to treat Homocystinuria?
Pyridoxine B6 **with** Folate B9
