Heme/Onc Flashcards by Jessica LaRosa

pRBC

1 unit raise Hb 3 points

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FFP

Replaces clotting factors
elevated PT, aPTT, INR
FFP is used as a replacement with plasmapheresis

NO FFP in IgA deficiency (must be IgA deficient donor)- anaphylactic reaction

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Cryopreciptate

Replace fibrinogen
High levels of Factor VIII and vWF
Useful in DIC
NEVER the first choice

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Sideroblastic anemia

Micro/Macrocytic 
Most accurate test: Prussian blue stain for ringed sideroblasts. Basophilic stippling 
Myelodysplasia 
Alcohol's suppressive effects on BM 
Lead poisoning 
INH 
Pyroxidine deficieny

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Microcytic anemia

low ferritin on iron study

Iron deficiency

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Microcytic anemia and high iron

Sideroblastic anemia

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Microcytic anemia and normal iron studies

Thalassemia

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Most accurate test for B-thalssemia

Hb electrophoresis

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Most accurate test for A-thalessemia

genetic studies

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Alpha thal

One gene deletion- nomral

two gene deletion- mild anemia, normal electrophoresis

three gene deletion- moderate anemia with HbH (beta tetrads)

Four gene deletion- gama-4 tetrads (HbBart), CHF and death in utero

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Beta thal

One gene deletion: Increased HbF and HbA2

Two gene deletion: beta thal intermedia, normal HbF, no transfusion dependence

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Differentiating between folate and B12 deficiency?

B12- elevated methylmalonic acid

Both will have elevated homocysteiene levels

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How to diagnose pernicious anemia?

Anti-intrinsic factor and anti-parietal cell Ab

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Sickle cell mutation

Point mutation at position 6 in beta chain-valine replaces glutamic acid

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Parvovirus B19 with sickle cell

Aplastic crisis
Look for sudden drop in retic count

Most accurate test: Parvovirus B19 PCR for DNA

TX: IVIG

(BM Bx-giant pronormoblasts)

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Causes of Warm/IgG hemolysis

CLL
Lymphoma
SLE
Penicillin, alpha-methyldopa, rifampin, and phenytoin

Causes of Cold/IgM hemolysis

EBV
Waldenstrom macroglobulinemia
M. pneumoniae

Best initial test for G6PD

Smear: Heinz bodies (methylene blue) and bite cells

Most accurate: G6PD level 1-2mo after acute episode

Pathophys of PNH

Clonal stem cell defect with increased sensitivity of red cells to complement in acidosis

Mutation of PIG-A gene- leads to deficiency in CD55 and CD59 (decay accelerating factors) cause over activation of complement while sleeping

Test by decreased CD55 and CD59 activity

Treat with Prednisone

Treatment for aplastic anemia

Transfusion/abx/platelets

Allogenic bone marrow transplant

Too old for BM transplant: antithymocyte globulin and cyclosporine/tacrolimus

Myelofibrosis

Pancytopenia associated with bone marrow fibrosis

Blood production in spleen and liver- tear drop shaped cells and nucleated RBC

Talidomide and lenalidomide- TNF inhibitors, increase BM production

AML M3

15:17, most associated with Auer rods (eosinophilic inclusions)
DIC, initial presentation

Treatment for AML/ALL

Chemotherapy first- remove blasts

Bad prognosis=bad cryogenics=immediate BMT

Good prognosis=good cryogenics=more chemo

ATRA for M3/promyelocytic leukemia

Add intrathecal MTX to ALL to prevent relapse in CNS

Persistently high WBC-neutrophils
Pruritus
Spenomeagly- early satiety, abdominal fullness, LUQ pain

CML

Dx: low leukocyte alkaline phosphatase score

Most accurate test: BCR-ABL (9:22, Philadelphia chromosome) by PCR or FISH

Tx: Tyrosine kinase inhibitors- imatinib

BMT curative

Most likely to transform into blast crisis.

Myelodysplastic syndrome

preleukemic condition Associated with 5q deletion (better prognosis) Pancytopenia with hyper cellular marrow Increased MCV, nucleated RBC, small number for blasts Ringed sideroblasts Tx: Transfusions EPO Lenalidomide-especially in 5q deletion

Elevated WBC in person >50yo Fatigue Lympahdenopathy Hepatosplenomeagly Infection

CLL ``` Dx: Elevated WBC- predominantly lymphocytes Hypogammaglobulinemia Anermia or thrombocytopenia- warm IgG/ BM infiltration Smudge cells on smear ```

CLL treatment

0/elevated WBC or 1/lymphadenopathy- no treatment 2/hepatosplenomagly, 3/anemia, 4/thrombocytopenia- fludarabine (purine analog) Refractory cases- cyclophosphamide Mild cases- chlorambucil (mustard gas/alkylating agent) Severe infection- IVIG Autoimmune thrombocytopenia/hemolysis- prednisone PCP prophylaxis in CML

Pancytopenia Massive splenomeagly Inaspiratly dry tap- hypercellularity

Hairy cell leukemia Dx: Initial- Smear showing hairy cells Best- flow cytometry for CD11c Tx: clardribine and pentostatin

Painless lymphadenopathy involving pelvic, retoperitoneal, or mesenteric stuctures Lymph nodes not warm, red, or tender Fever, weight loss, drenching sweat

Non-hodgkin lymphoma Dx: initial- excisional bx CBC normal Stage: CT C/A/P and BM Bx

NHL treatment

1-lymph node group 2- 2+ lymph node groups on same side of diaphragm 3- both sides of diaphragm 4- widespread disease 1+2- local radiation and small course chemo ``` 3+4+any B sx- CHOP and rituximab C-cylchophosphamide H-adriamycin O-vincristine P-prednisone ```

Hodgkin lymphoma treatment

A-adriamycin- nuclear ventriculogram prior to determine EF B-bleomycin V-vinblastine D-darbazine

Doxirubicin toxicity

Cardiomyopthat get MUGA or nuclear ventriculogram first

Vincristine toxicity

Neuropathy

Bleomycin toxicity

Lung fibrosis

Cyclophosphamide

Hemorrhagic cystitis

Cisplatin

Renal toxicity and ototoxicity

Diagnosis of Multiple Myeloma

X-ray of affected bone showing lytic lesion (radionuclide scan is normal in lytic lesions) Serum electrophoresis IgG or IgA spike Most accurate: >10% BM with plasma cells

``` Lethargy Blurry vision, vertigo Engorged blood vessels in the eye Mucosal bleeding Raynaud phenomenon ```

Waldenstrom Macroglobulinemia Dx: IgM spike on SPEP (cause hyperviscosity Tx: plasmapheresis