Path: - Autosomal recessive - HgbSS - Valine --> glutamine - Sickling under stress (acidosis, dehydration)

OMED Hematology Oncology Flashcards by Andrew Pruett

Folate deficiency
Path:
Pt:
Dx:
Tx:

Path: leafy green, 3-6 week stores, pregnancy
Pt: tea+toast diet, EtOh, pregnancy
Dx: Folic acid levels decreased, but normal methyl malonic acid
Tx: folate, 1 mg po

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B12 deficiency 
Path:
Pt:
Dx:
Tx:
Fu:

Path: 3-10 yr storage, animal products, neuro symptoms
Pt: vegan, pernicious anemia, crohn’s, gastric bypass
Dx: B12 levels decreased, increased methyl malonic acid
Tx: B12, PO= nutritional, IM= impaired absorption
Fu: never choose schilling test on exam, prolonged deficiency causes DCMLS

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Pernicious Anemia 
Path:
Pt:
Dx:
Tx:
fu:

Path: Antibodies against parietal cells, no intrinsic factor, no B12 absorption

Pt: weakness, sore tongue, paresthesias

Dx: Ab-intrinsic factor, Ab-anti parietal
Tx: IM B12, cannot treat pernicious anemia
F/u: gastric cancer

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non megaloblastic anemia 
Path:
Pt:
Dx:
Tx:

Path: liver diz, EtOh, medications: (AZT, HAART, 5 FY, ARA-C), metabolic syndromes (Lesch Nyhan, Hereditary orotic aciduira)

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Iron deficiency anemia 
Path:
Pt:
Dx:
Tx:

Path: slow bleed, consumption of iron stores
Pt: women- menorrhagia, older men = colon cancer
Dx: iron studies- ↓iron, ↑TIBH, ↓ Ferritin
Tx: iron 2-6 wks for anemia, iron 2- months for iron stores

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Anemia of chronic disease 
Path:
Pt:
Dx:
Tx:

Path: chronic inflammation
Pt: anemic
Dx: iron studies: iron studies- ↓iron, ↓TIBH ↑ Ferritin. bone marrow biopsy, rarely needed.
Tx: treat underlying condition, EPO for severe cases

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Thalassemia 
Path: 
Pt:
Dx:
Tx:

Path: genetic mutations, loss of genes, 4 alpha, 2 beta 
Pt: 
-Asx: 1 alpha deletion (asymptomatic)
-Minor: 2 alpha deletions, 1 beta deletions 
-Major: 3 alpha deletions, 2 beta deletions 
-Dead: 4 alpha deletions 
Dx:
- Fe normal
- Ferritin normal
- TIBC normal 
- Hgb Electrophoresis 
Tx: 
-minor: do nothing 
-major = transfusions = deferoxamine

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sideroblastic anemia 
path:
Pt:
Dx:
Tx:

Path:
-irreversible: B6, cancer
-reversible: lead, EtOh, copper, isoniazid
Pt: anemia
Dx:
-Fe ↑, Ferritin normal, TIBC normal
-BEST: bone marrow biopsy showing ringed sideroblasts
Tx: remove exposure, give back B6, try to treat cancer

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Sickle Cell

Path:

Autosomal recessive
HgbSS
Valine –> glutamine
Sickling under stress (acidosis, dehydration)

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Sickle Cell

Pt:

Pt:
Emergency acute-
*acute chest = MI, CHF
*acute brain = CVA 
*priapism

Hospitalized Acute-
*vaso-occlusive crisis

Chronic-

asplenia
avascular necrosis
osteomyelitis

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Sickle Cell
Dx:
Tx:
F/u

Dx:

*1st- smear = sickled cells –> used on first crisis, not subsequently. if they were diagnosed previously, dont use smear.
*
Best: Hgb electrophoresis (Sc, SS)
-use 1st time only

Tx: hydroxyurea = ↑HgbF, ↓HgbSS, IVF, pain control, exchange transfusion in emergency
f/u: iron overload (deferoxamine)

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G6PD deficiency
Path:
Pt:
Dx:
Tx:

Path: ↓ G6PD, hypoxemic = hemolysis
Pt: African American Males, dapsone, TMP-SMX, nitrofurantoin
Dx: 1st smear = bite cells, heinz bodies.
best: G6PD levels 6-8 weeks
Tx: supportive, avoid stressors

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Hereditary spherocytosis 
Path:
Pt:
Dx:
Tx:

Path: deficiency in spectrin, ankyrin, pallidin
Pt: hemolysis, spherocytosis
Dx:
smear–>spherocytes –> osmotic fragility test (+)
Tx: folate + Fe, splenectomy

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Paroxysmal Nocturnal Hemoglobinuria 
Path:
Pt:
Dx:
Tx:

Path: PIGA deficiency 
Pt:
Paroxysmal (once in awhile)
Nocturnal (only at night)
Hemoglobinuria (dark urin)

Dx: flow cytometry, ↓CD55, ↓CD59
Tx: Biologics (Eculizumab)

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Warm Autoimmune Hemolytic Anemia 
Path:
Pt:
Dx:
Tx:

Path: IgG (Cancer,Drugs,Rheumatoid)
Pt: Hemolysis Everywhere
Dx: Coombs Test IgG
Tx: 
-First line: steroids
-Recurrent: splenectomy
-Severe: IVIg
-Refractory to splenectomy: rituximab

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Cold autoimmune hemolytic anemia
Path:
Pt:
Dx:
Tx:

Path: IgM (Mycoplasma, mono)
Pt: Hemolysis in the cold
-tips of digits and nose
Dx: First line: Coombs test negative for IgG
Tx: 
-Avoid the cold
-Refractory: rituximab

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Microangiopathic Hemolytic anemia
Path:
Pt:
Dx:
Tx:

Path: Schistocytes = MAHA
Pt: TTP versus DIC
Dx: 
-Decreased hemoglobin (both)
-Decreased PLT (both)
-Decreased fibrinogen (DIC)
-Increased INR (DIC)
-Increased split products (DIC)
Tx: See thrombocytopenia

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Acute myelogenous leukemia
Path:
Pt:
Dx:
Tx:

Path: 
acute = Blasts
Myelogenous= Neutrophils
Leukemia= Cancer in the blood
Pt:
-Acute, age 67
-Exposure: Benzene radiation
-CML: Blast crisis
Dx: 
-First: Smear= blasts
-BM Bx > 20%
Tx: 
M3: Vit A )
auer Rods on bx (-) M3: chemo

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Acute lymphocytic leukemia
Path:
Pt:
Dx:
Tx:

Path:
-acute = blasts 
-lymphocytic = leukocytes 
-leukemia = cancer in the blood
Pt: acute, age 7
Dx: 
-First: smear = blasts
-BM Bx > 20% blasts + cALLa and TdT
Tx:
-chemo
-PPx CNS ARA-C +/- radiation

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Chronic myelogenous leukemia

Path:

Chronic =mature cells
Myelogenous =neutrophils
Leukemia =cancer in the blood

pt: Chronic, age 47

Dff (way more cells then should be)
BM Bx = Philadelphia + t(9, 22) + BCR – ABL

tx: Imatinib (tyrosine kinase)

f/u: blast crisis

Chronic lymphocytic leukemia

Path:
Pt:
Dx:
Tx:

path:
- Chronic equals mature cells
- Lymphocytic equals lymphocytes
- Leukemia equals cancer in the blood

pt: Chronic, Age 87
dx: diff (Way more cells that should be)

tx:
- 65+ and asx = nothing
- 65+ and Sx = chemo
- < 65 + donor = HSCT

Contiguous lymph node spread, B sxs, painful lymphadenopathy with EtOh

Pt:
Dx:
Tx:

Hodgkin’s lymphoma

Pt: nontender LAD
Dx:
-excisional biopsy showing Reed Sternberg Cells
-Staging with PET/CT or CT Chest/Abdo/Pelvis

Tx:
IIa or better = radiation
IIb or worse = chemo (ABVD, BEACOPP)

Hematogenous spread
usually no B sxs
starry sky on biopsy
Extranodal diz

Pt:
Dx:
Tx:

NHL

Pt: nontender LAD +/- sxs

Dx:

excisional biopsy
NO reed sternberg cells
stage PT/CT or CT chest/abdo/pelvis

Tx:

IIa or better = chemo
IIb or worse, chemo = ABVD or BEACOPP

Staging of lymphoma I-IV

LNs location relative to diaphragm

I = I = not applicable 
II = 2+ and same side diaphragm 
III = 2+ and opposite side of diaphragm 
IV = Mets = N/A

Adverse Effects ``` cisplatin- bleomycin- adriamycin/doxorubicin- vincristine/vinblastine- cisplatin- cyclophosphamide- ```

cisplatin- ototoxicity bleomycin- pulmonary fibrosis adriamycin/doxorubicin- cardiac toxicity vincristine/vinblastine- peripheral neuropathy cisplatin- nephrotoxicity cyclophosphamide- hemorrhagic cystitis

70 yo with hypercalcemia, renal failure, anemia, and bone pain ``` Path: Pt: Dx: Tx: f/u ```

Multiple myeloma Path: IgG, monoclonal expansion of plasma cells - Ig = recurrent infections - bence jones = AKI - osteoclasts = lytic lesions Pt: 70s+ male with CRAB symptoms (hypercalcemia, renal failure, anemia, and bone pain) Dx: Spep+ Upep+ Skeletal survey + BM Bx > 10 % plasma cells Tx: above 70 - chemo, no donor. below 70, donor: HSCT F/u: melphalan + steroids, thalidomide, bortezomib

Incidental IgG spike without CRAB symptoms Path: Pt: Dx: Tx:

MGUS, early myeloma Path: increased IgG Pt: 85+ yo, no CRAB symptoms Dx: Spep+, Upep- , skeletal survey - , BM Bx < 10% plasma cells Tx: watch and wait, converts to MM 2%/yr

Constitutional symptoms, hyperviscosity syndrome, IgM Pt: Dx: Tx: f/u

Waldenstrom's Path: IgM Pt: Constitutional symptoms, hyperviscosity syndrome Dx: Spep + , Upep - , skeletal survey - , BM Bx > 10% lymphoma Tx: -Rituximab - chemo -Hyperviscosity = plasmapheresis F/u: blast crisis

Thrombophilic Disorders listed

``` Factor V Leiden (mutation) Prothrombin 20210A (mutation) Protein C def (level) Protein S def (level) Antithrombin def (level) ```

Who gets warfarin in a DVT?

All patients except metz cancer pts "provoked 3 months" "unprovoked 6 months" recurrence lifetime

DVT prophylaxis risk cancer

risk: - trauma - surgery (10x) - LWMH PPx - LMWH --> warfarin Cancer LMWH ppx LMWH treatment (no warfarin in met cancer)

fever, anemia, thrombocytopenia, renal fialure, neuro symptoms Path: Pt: Dx: Tx:

TTP ``` Path: hyaline clots, adamts-13 deficiency Pt ("FAT RN") Fever Anemia Thrombocytopenia Renal failure Neurological symptoms ``` Dx: - CBC: ↓ platelets - Smear: schistocytes - PT/PTT: normal - fibrinogen: normal - D-dimer: normal Tx: -exchange transfusion F/u: NEVER platelets

SICK AS SHIT, SEPSIS, ICU SHOCK, THEN THEY START BLEEDING Path: Pt: Dx: Tx:

DIC Path: fibrin clots, tremendous catastrophic injury Pt: SICK AS SHIT, SEPSIS, ICU SHOCK, THEN THEY START BLEEDING Dx: - CBC: ↓ platelets - Smear: schistocytes - PT/PTT: ↑ - fibrinogen: ↓ - D-dimer: ↑ Tx: - Fix underling disease - give blood products

thrombocytopenia following heparin exposure Path: Pt: Dx: Tx:

HIT Path: antibodies to platelets Pt: heparin --> (7-14 days)--> Plts ↓ by ~50%--> new clots Dx: antiplatelet factor 4 Elisa +, confirm with serotonin release assay Tx: stop heparin, argatroban --> warfarin INR 2-3

Woman with autoimmune disease and ↓ platelets Path: Pt: Dx: Tx:

Path: antibodies to platelets, splenic destruction Pt: Woman with autoimmune disease and ↓ platelets Dx: Diagnosis of exclusion Tx: steroids, IVIg, splenectomy, rituximab

woman with heavy menstrual periods and easy bleeding Path: Pt: Dx: Tx:

Path: - MC bleeding disorder - Deficiency of vWD - autosomal dom Pt: platelet bleed, normal count, can have factor VIII def Dx: - 1st - CBC = normal count - best = vWD assay - must have ↑ PTT from factor VIII deficiency Tx: DDAVP (Desmopressin)

Bleeding AND clotting, hemarthrosis (bleeding), arterial thrombosis (clotting) Path: Pt: Dx: Tx:

Antiphospholipid antibody syndrome Path: LUPUS Pt: Bleeding AND clotting, hemarthrosis (bleeding), arterial thrombosis (clotting) Dx: Pt/PTT/INR, mixing studies that do not correct, Vpier Venom Assay Tx: heparin to warfarin bridge for APLA, treat lupus

``` Acquired Factor Deficiency Path: Pt: Dx: Tx: ```

Path: liver dysfunction, warfarin, Vit K def Pt: long ICU stay, on warfarin, has cirrhosis Dx: PT/PTT/INR Tx: Vitamin K

Bleeding Disorders: list them

``` VWD ITP TTP DIC Glanzmann's Bernard-Soulier Hemophilia Liver Disease Vit K Deficiency ```

Thrombophilia (over-clotting) diseases: list them

``` Factor V Leiden Protein C Deficiency Protein S Deficiency Antithrombin Antiphospholipid Syndrome ```

gingival, vaginal, skin bleeding are signs of a problem in which ° of hemostasis?

primary hemostasis = platelet bleeding normal platelet count = platelet function error low platelet count = platelet destruction/consumption (thrombocytopenia)

primary hemostasis with a normal normal platelet count = primary hemostasis with a low platelet count =

primary hemostasis = platelet bleeding normal platelet count = platelet function error low platelet count = platelet destruction/consumption (thrombocytopenia)

main causes of thrombocytopenia

A. destruction (ITP, TTP, DIC, HIT) B. production (aplastic, mylodysplastic syndromes, cancer) C. sequestration - splenic dysfunction or liver disease

Bernard Soulie vs Glanzmann's

BS has one b: problem is Glycoprotein I B so GT Glycoprotein II B 3A

PT/PTT abnormalities are signs of a problem in which ° of hemostasis?

``` secondary hemostasis (factors) THEN PERFORM ``` ``` -mixing study ↓ bleeding continues- indicates presence of inhibitor BUT IF ↓ -mixing study: BLEEDING TIME CORRECTED--> Hemophilia A/B, Vitamin K def, Warfarin therapy DIC, WBD (Factor VIII instability) ```

``` Causes of a primary hemostasis dysfunction with a normal platelet count = ```

NSAIDs, ASA, clopidigrel | uremia, VWD

Causes of a primary hemostasis dysfunction with a low platelet count =

Destruction of platelets (ITP, TTP, HTP, DIC) Underproduction of Platelets (myelodysplastic syndromes, cancer) Sequestration of platelets (splenic dysfunction and liver disease)

How to diagnose a bleeding disorder

CBC PT/PTT (then mixing study if abnormal) Platelet count- normal or low? Hemoglobin- normal or precipitous drop?