Heme/Onc Flashcards
pRBC
1 unit raise Hb 3 points
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
Cryopreciptate
Replace fibrinogen
High levels of Factor VIII and vWF
Useful in DIC
NEVER the first choice
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
Microcytic anemia
low ferritin on iron study
Iron deficiency
Microcytic anemia and high iron
Sideroblastic anemia
Microcytic anemia and normal iron studies
Thalassemia
Most accurate test for B-thalssemia
Hb electrophoresis
Most accurate test for A-thalessemia
genetic studies
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
Beta thal
One gene deletion: Increased HbF and HbA2
Two gene deletion: beta thal intermedia, normal HbF, no transfusion dependence
Differentiating between folate and B12 deficiency?
B12- elevated methylmalonic acid
Both will have elevated homocysteiene levels
How to diagnose pernicious anemia?
Anti-intrinsic factor and anti-parietal cell Ab
Sickle cell mutation
Point mutation at position 6 in beta chain-valine replaces glutamic acid
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)
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
