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
Most common adult NHL
Diffuse
(11;14) cyclin D, CD23-
Mantle cell
- Cyclin promotes G1–>S
(14;18) bcl-2, CD10+, CD5-
Follicular
- prevents apoptosis
- Tx = Rituximab CD20 Ab
CD20+, assoc w/ auto-immune diseases/chronic inflammatory - Hasimoto’s, Sjogren, H. pylori
Marginal zone
- MALTOMA - H. pylori
Most common primary malignancy of bone
Multiple myeloma
High IL-6, bone pain w/ hyperCa, M-spike of IgG on SPEP, Rouleaux formation, clock-face chromatin
Multiple myeloma
- Bence-Jones proteins –> renal failure
- Light chain amyloidosis
- M-spike = IgG or IgA
- Lytic bone lesions
Bence-Jones Proteins
Multiple myeloma
M-spike w/ IgM, retinal hemorrhage, papilledema, bleeding from nose and gums, no lytic bone lesions or renal impairment, HA
Waldenstroms
- Tx = plasmaphoresis
MGUS = Asx + <10% plasma cells on BM Bx
10 y/o w/ infections, fatigue, bone pain, petechiae, bleeding Down >5 CNS, testicular, LA Tdt + (9;22) Lymphoblasts
ALL
- B-type most common
- CALLA Ag
65 y/o Down <5 Auer rods MPO & Sudan black stains Myeloblasts (15;17)
AML
- M3 = ATRA, Auer rods –> DIC, 15;17
- M5 = no MPO, GUMS
- M7 = Down <5
Auer rods
AML
(9;22)
CML & ALL
(11;22)
Ewing sarcoma
(15;17)
M3 type of AML (all trans retinoid acid)
T-ALL
Tdt CD2, 8 NO CD10 Thymic mass Teens (remember all the Ts)
CD5, CD20, CD23+
Smudge cells
>60 y/o
CLL
Stains with tartrate resistant acid phosphatase, splenomegaly, dry bone aspirate, fried egg appearance
Hairy cell leukemia
- All the TRAPs
- Tx = 2-CDA
Cerebriform nuclei, CD4 and CD3 cell aggregate in epidermis (Pautrier microabscesses)
Mycosis fungiodes
30-60
(9;22) bcr-abl
(-) Leukocyte alkaline phosphatase
CML
- Tx = imatinib (TK inhibitor for bcl-abl)
- 2/3 –> AML
- 1/3 –> ALL
Myeloproliferative disorders assoc w/ JAK mutations
Polycythemia
Thrombocytosis
Myelofibrosis - tear-drop cell
Heparin
Activates AT-III
Ok for preggers
Enoxaparin
LMW
- more predictable, low HIT
- selective Xa inhibitor
Direct thrombin inhibitors
Lepirudin
Bivalrudin
Fondaparinux
Binds only AT-III
DVT prophylaxis*
Clopidogrel, ticlopidine
Block ADP receptors –> inhibit platelet aggregation
Ticlopidine –> neutropenia
Cilostazol, dipyridamole
PDE III- inhibitor –> inc cAMP –> inhibits platelet aggregation
Used for claudication, prevention of stroke
Abciximab
GPIIb/IIIa inhibitor
Inhibits M-phase
Vinca alkyoids & paclitaxol
Bind tubulin preventing spindle formation
Colchicine, Vincristine, Vinblastine
- Block polymerization in M-phase
Bind microtubules preventing DEploymerization
Paclitaxel
Inhibits topoisomerase
Etoposide
Alkylating agents
Cisplatin
DNA intercalators
Dactinomycin
Doxorubicin
Inhibits thymidylate synthase
MTX
5-FU
Dec purine synthesis
6-MP
- Inc toxicity w/ allopurinol
- 6-TG ok w/ allopurinol
MTX antidote for myelosuppression
Leucovorin (folinic acid)
Used for kids tumors
ACTinomycin D - intercalates
DNA intercalator assoc w/ cardiotoxicity
Dox and danrubicin
Used in brain tumors
Nitroureas - carmustin, lomustine, semustine
Croses BBB
Inhibits cycophilin to inhibit T-cell activiation
Cyclosporine
Inhibits immunophilin FKBP
Tacrolimus
Inhibits FKBP-12, m-TOR, T-cell response
Sacrolimus
Inhibits CD52 in lymphoma
Alemtuzumab
Inhibits VEGF in Colorectal cancer
Bevacizumab
Inhibits CD33 in AML
Gemtuximab
Inhibits EGFR in colorectal cancer
Cetuximab
Drugs cause peripheral neuropathy, BM suppression
Vincristine
VinBlastine = BM
Drugs cause nephro and ototoxicity
Cisplatin, carboplatin
Drug specific for S-phase inhibit RnR
HU
Monclonal Ab to HER-2/neu
Traztuzumab
Dapsone
Inhibits dihydropteroate synthase
Used for Malaria, Toxo, M. leprae
can –> hemolysis in G6PD
Hyperproliferation of megakaryocytes, thrombocytosis,
Essential thrombocytosis
- JAK2 mutation
- Tx = aspirin
VTs and skin necrosis w/ warfarin use
Protein C deficiency
Inc Hct, WBCs, platelets
Vertigo, HA, tinnitus, thrombosis, gout, digital ischemia, CVA/MI/DVT/PEs, PRURITIS AFTER SHOWER, Erythromelagia
- Polycythemia vera*
- JAK2 mutation
- Erythromelagia = red burning extremities relived by cold and aspirin
Splenomegaly, mild anemia, dry bone marrow aspirate, tear-drop cell
Myelofibrosis
- JAK2 mutation
- Fibrosis of BM
Pt being treated for CML has developed pulmonary fibrosis and hematuria is most likely on what drug?
Busulfan
- Alkylating agent –> N7 of guanosine
Functions to bind factor VIII and prevent degradation by protein C, deficiency presents with excessive epistaxis, menorrhagia
vWF disease
- Tx = DDAVP –> retains water –> hypoNa, K
Anaphylaxis after blood transfusion
IgA deficiency
7 y/o w/ short stature, cafe au lati, short thumb, pancytopenia
Fanconi anemia
- DNA repair genes