Heme/Onc Flashcards
Dark urine in the morning that improves throughout the day, + sucrose test
PNH
- precipitated by iron, vaccines, infections
- Inc COMPLEMENT destruction d/t impaired GPI anchor
Osmotic fragility test +
Hereditary spherocytosis
- spectrin, ankyrin gene
- aplastic crisis
Cigar-shaped RBCs
Hereditary elliptiocytosis
+ coombs, normocytic anemia
Auto-immune hemolytic anemia
Back pain with hemoglobinuria a few days later, Heinz bodies, Bite cells
G6PD deficiency
- sulfas, anti-malarials, fava beans
Burr cells/echinocytes/acanthocytes
Pyruvate kinase deficiency
Babesia DOCs
Quinine
Clindamycin
Metabolite of cyclophosphamide responsible for hemmorrhagic cystitis
Acrolein
- mercaptoethansulfate (mensa) binds this
Drug used in 9:22 translocation leukemia
Imatinib used in CML
- Inhibits TK activity from BCR-ABL fusion
Inc TIBC
Dec, ferritin, Serum Fe, % sat, free protoporphyrin
Iron deficiency anemia
- Duodenctomy, Breast fed, Ulcers, Pregnancy, Polyps, Hookworm
Inc Ferritin, protoporphyrin
Dec TIBC, Serum Fe, % sat
Anemia of chronic disease
Iron-laden mitochondria
Inc Ferritin, Serum Iron % Sat
Dec TIBC
Sideroblastic anemia
- aminolevulinic acid synthase deficiency (ALAS)
- alcohol, lead, B6 deficiency
- Tx = pyridoxine (B6)
Pale line on gingiva, basophilic stippling of RBCs, abdominal colic, siderblastic anemia
Lead poisoning
- Inhibits ferrochelatase & ALA dehydratase
- Tx = Dimercaprol + EDTA
- Kids = Succimer
Alpha-Thal
1-2 deletions = no Sx
3 deletions = HbH (B4)
B-Thal Minor (B/B+)
Usually ASx
HbA2 >3.5%
B-Thal Major (B0/B0)
ABSENT B chain –> (a2, y2)
- Ineffective erythoporesis
- Erythroid hyperplasia –> crew cut, chipmunk facies
- Extramedullar hematopoesis –> nucleated RBCs
- Aplastic crisis
- No HbA, inc HbA2, HbF
Anemia w/ >3% corrected Retic count, inc LDH, inc unconjugated bilirubin & jaundice, no hemoglobinuria, bile stones, splenomegaly
Extravascular hemolysis
- Spherocytosis
- Sickle Cell
- Pyruvate kinase
- HbC
- G6PD
Anemia w/ >3% corrected Retic count, inc LDH, dec haptoglobin, hemoglobinuria
Intravascular hemolysis
- PNH
- G6PD
- Immune hemolytic
- Microangiopathic
- Malaria
Howell-Jolly bodies
Hereditary spherocytosis after splenectomy
HbC defect
Glut –> Lysine
Sickle Cell defect
Glut –> Valine
- Low O2, or dehydration –> inc sickling
- Extramedullary hematopoesis
- AUTOSPLENECTOMY
- dactylitis in kids
- HU –> inc HbF
SLE CLL, methyldopa, warm IgG anemia, Coombs +
Warm agglutinin auto-immune hemolytic anemia
Triggered by cold, mycoplasma, Mono, CLL, IgM, Coombs +
Cold agglutinin auto-immune hemolytic anemia
Helmet cells on blood smear (schistocytes)
Microangiopathic
- DIC, TTP-HUS, SLE, HTN, prosthetic valves
Dec TIBC
Hemochromatosis
Urine that turns tea color when exposed to light
PCT
- Uroporphyrinogen decarboxylase
Painful abdomen, Red-wine colored urine, polyneuropathy, psych disturbances
Acute intermittent porphyria
- Defect in porphobilinogen deminase
Dec GP1b
Bernard-Soulier
- can’t bind vXF
Dec GPIIb/IIIa
Glansmann’s
- Defect in platelet-platelet
Anti-GPIIb/IIIa Abs
Idiopathic thrombocytopenic purpura
- platelet-AB complex consumed by MO
ADAMTS 13 deficiency
Thrombocytopenic purpura (TTP) - LARGE vWF multimers --> thrombosis
Most common inherited bleeding disorder
vWF disease
- Tx = DDAVP
Most common inherited hypercoagulability
Factor V Leiden
- Can’t be degraded by protein C
Reed-sternberg cells (bilobed nuclei w/ prominent nucleoli surrounded by clear cytoplasm)
Hodgkin lymphoma
- CD15, 30+
- CD20 -
- Tx = brentuximab (CD30 Ab)
Young female, bands of sclerosis, lacunar cells, enlarging LN
Nodular sclerosing HL
Best prognosis HL
Lymphocyte rich
Worst prognosis HL
Lymphocyte depleted
(8;14) c-myc, starry sky
Burkitt’s
- CD20+
- EBV
- africa = Jaw
- sporadic = abdomen