Heme Flashcards
Why are blood smears not effective for microcytosis
All are hypo chromic and can have target cells
What patients with anemia are transfused?
Symptomatic or low hct in elderly or heart diseased
Low ferritin microcytosis?
Iron deficiency
High iron microcytosis?
Sideroblastic
Normal iron microcytosis?
Thalassemia
Iron deficiency anemia unique lab features?
Increased rdw and increased platelet count
Sideroblastic unique lab features
Ringed blasts with Prussian blue
Thalessamia diagnosis test
Electrophoresis
First step in distinguishing macrocytic anemia
Peripheral blood smear to detect hypersegmented neutrophils
Classic neuropathy of b12 deficiency
Posterior column damage to position and vibratory sensation possibly causing ataxia
What test distinguishes b12 and folate deficiency
Mma is elevated in b12 only
What is a complication of b12 or folate deficiency
Hypokalemia
Sickle cell mutation?
Point mutation at position 6 of beta globin changes glutamic acid to valine
Diagnostic tests in scd
Initial: peripheral smear
Accurate: hb electrophoresis
Where do you see Howell jolly bodies
Scd
What lowers mortality in scd
Hydroxyurea + Abx with fever
Vaccine for asplenia in scd?
Pneumococcal
Test for aplastic crisis in scd? And tx?
Initial: reticulocyte count
Accurate: pcr for parvo b19 dna
Treat: IVIG
Treat hereditary spherocytosis
Chronic folic acid + splenectomy
Treating AIHA
Warm: steroids –> splenectomy –> IVIG –> Rituximab
Cold: stay warm –> Rituximab –> other immunosuppressive
G6pd presentation
African or Mediteranean male with sudden anemia after infection or dapsone/quinidine/sulfa/primaquine/nitrofurantoin/fava beans
Initial: Heinz bodies
Accurate: test g6pd level 1 to 2 months after hemolysis
What disease is worsened by platelet transfusion
Ttp and hus
PNH cause of death and treatment
Thrombosis (mesentaric or hepatic V)
Tx: steroids –> BM transplant (cure) –> Eculizumab to inactivate C5
Inhibitor of JAK2
Ruxolitinib
AML (4 things)
M3 gene (causes DIC, treat with ATRA)
Auer Rods
cr1517
myeloperoxidase
Persistently elevated WBC count of all neutrophils
CML
CML test + initial tx + cure
Accurate test: BCR-ABL (922) on PCR or FISH
Tx: TK inhibitor (imatinib)
Cure: BMT = never 1st therapy!
Leukostasis rxn 1st step
Leukapheresis
Classic abnormality of MDS
5q deletion
Distinct lab abnormality of MDS
Pelger Huet cells (bilobed nucleus)
Treating MDS
Lenalidomide for 5q deletion to decrease transfusion dependence
Azacitidine also decreases transfusions and increases survival
Treating CLL
Stage 0 (elevated WBC): None Stage 1 (lymphadenopathy): none Stage 2 (hepatosplenomegaly): none Stage 3 (anemia), stage 4 (thrombocytopenia): fludarabine + cyclophosphamide + Rituximab Fludarabine failure: Alemtuzumab Refractory cases: Cyclophosphamide Mild cases in elderly: Chlorambucil Severe infection: IVIG AIHA/thrombocytopenia: prednisone
Accurate test for hairy cell leukemia
Immunotyping by flow cytometry
Treat hairy cell leukemia
Cladrabine or Pentostatin
NHL initial test and severity correlation
excisional biopsy (NOT needle aspiration) High LDH indicates worsening severity
Staging NHL
1: lymph node group
2: 2 or more nodes on 1 side of diaphragm
3: both sides of diaphragm
4: widespread disease
NHL tx
Local (1a/2a): local radiation and small chemo dose
3+: CHOP (Cyclophosphamide/Adriamycin/Vincristine/Prednisone) + Rituximab
HL
RS cells
Lymphocyte predominant is good for prognosis
Treating stage 3 or 4 HL
ABVD: Adrimycin (doxorubicin)/Bleomycin/Vinblastine/Dacarbazine
Radiation complications
Cancer (screen 8 years later), CAD
What does the EF need to be for Adrimycin and how do you screen
> 50%
MUGA or nuclear vetriculogram
4 presenting factors of Multiple Myeloma
Bone pain
Hyperuricemia (high turnover)
Anemia (bony infiltration)
Renal Failure (accumulated IG and Bence Jones)
Diagnostics of MM
Punched out lyric lesions SPEP shows IgG or IgA spike HyperCa Bence JOnes Rouloux formation decreased AG due to IgG (cationic) increasing Cl and HCO3
Why does urinary level of protein stay low in MM
Dipstick only measures albumin
SPEP results
99% of those with an M spike do NOT have MM –> must do BM biopsy
Waldenstrom Macroglobinuria (pathophys/sx/tx)
Increased IgM causes hyper viscosity
Lethargy/engorged eye vessels/mucosal bleeding
Plasmapheresis initially
Bleeding disorders (platelet vs factor)
Superficial vs deep
Initial management of mild bleeding
Prednisone
Vaccines before splenectomy
Meningitis
H flu
Pneumococcus
VWD dx and tx
Dx: normal platelet count, worsened by ASA, Ristocetin +
Tx: DDAVP
Hemophilia dx
Delayed joint or muscle bleeding in a male child, prolonged PTT
Confirming HIT
ELISA for platelet factor 4 (PF4) ABs or the serotonin release assay
Tx for HIT after stopping heparin containing products
Administer direct thrombin inhibitors (Argatroban, Lepirudin, Biivalirudin, Fondaprinux) –> administer Warfarin after
Most common cause of hyper coagulation
Factor V Leiden
Tx for Anti Phospholipid Syndrome
Possibly lifetime anticoagulation
Initial evaluation of anemia
CNBC