Heme/Onc Flashcards
1
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- Acanthocyte (“spur cell”)
- Liver disease, abetalipoproteinemia (states of cholesterol dysregulation)
2
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- Basophilic stippling
- Lead poisoning, sideroblastic anemias, myelodysplastic syndrome
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- Dacrocyte (“teardrop cell”)
- Bone marrow infiltration
4
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- Degmacyte (“bite cell”)
- G6PD deficiency
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- Echinocyte (“burr cell”)
- End-stage renal disease, liver disease, pyruvate kinase deficiency
- Different from acanthocyte: projections more uniform and smaller
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- Elliptocyte
- Hereditary elliptocytosis, usually asymptomatic
- caused by mutation in genes encoding RBC membrane proteins (eg, spectrin)
7
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- Macro-ovalocyte
- Megaloblastic anemia (also hypersegmented PMNs)
8
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- Ringed sideroblast
- Sideroblastic anemia; Excess iron in mitochondria
9
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- Schistocyte
- Microangiopathic hemolytic anemias, including DIC, TTP/ HUS, HELLP syndrome, mechanical hemolysis (eg, heart valve prosthesis)
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- Sicklecell
- Sickling occurs with dehydration, deoxygenation, and at high altitude
11
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- Spherocyte
- Hereditary spherocytosis, drug- and infection-induced hemolytic anemia
12
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- Target cell
- HbC disease, Asplenia, Liver disease, Thalassemia (HALT the target)
13
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- Heinz bodies
- Seen in G6PD de ciency.
14
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- Howell-Jolly bodies; Basophilic nuclear remnants found in RBCs
- Seen in patients with functional hyposplenia or asplenia
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19
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20
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Anti-coag factors produced by endothelium
A
PGI2 and NO
21
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Ristocetin
A
Activates vWF to bind GpIb
Failure of aggregation in vW disease and Bernard-Soulier
22
Q
α-thalassemia
A
- 4 deleted: no α-globin; Excess γ-globin forms γ4 (Hb Barts): hydrops fetalis
- 3 allele deletion: very little α-globin. Excess β-globin forms β4 (HbH)
- 2 allele deletion: less clinically severe anemia
- 1 allele deletion: no anemia (clinically silent)
23
Q
β-thalassemia minor
A
β chain is underproduced; Usually asymptomatic.
Diagnosis confirmed by HbA2 (> 3.5%) on electrophoresis
24
Q
β-thalassemia major
A
β chain is absent severe microcytic, hypochromic anemia with target cells and increased anisopoikilocytosis
Marrow expansion, B19 infxn
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Lead poisoning
* inhibits ferrochelatase and ALA dehydratase
* decr heme and incr protoporphyrin
Symptoms of LEAD poisoning:
* Lead Lines on gingivae (Burton lines) and on metaphyses of long bones D on x-ray.
* Encephalopathy and Erythrocyte basophilic stippling
* Abdominal colic and sideroblastic Anemia
* Drops—wrist and foot drop
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Treatment for lead poinsoning
Dimercaprol and EDTA are 1st line of treatment
Succimer used for chelation for kids
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Sideroblastic anemia causes
X-linked defect in ALA synthase gene)
myelodysplastic syndromes
reversible: (alcohol is most common; also lead, vitamin B6 de ciency, copper de ciency, isoniazid)
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Siderblastic anemia lab findings
Incr iron, decr TIBC, incr ferritin
Ringed siderblasts
Basophilic stippling
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Treatment for sideroblastic anemia
pyridoxine (B6, cofactor for ALA synthase).
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Folate deficiency
Malnutrition, malabsorption, drugs
Incr homocystein; nml methylmalonic acid
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Vit B12 deficiency
Vegetarian, Crohn, tapeworm
Incr homocystein and methylmalonic acid
SHilling test +
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Orotic aciduria
Inability to convert orotic acid to UMP
(de novo pyrimidine synthesis) d/t defect in UMP synthase
failure to thrive, developmental delay, and megaloblastic anemia refractory to folate and B12
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Diamond-Blackfan anemia
Rapid-onset anemia within 1st year of life due to intrinsic defect in erythroid progenitor cells.
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Hereditary spherocytosis
Extravascular hemolysis; defect in RBC membrane protins (ankyrin)
Small, round RBCs with less surface area and no central pallor ( MCHC)
+osmotic fagility test
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G6PD defeciency
Decr glutathione: increased susecptibility to oxidative stress
Blood smear shows RBCs with Heinz bodies and bite cells
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Pyruvate kinase defeciency
Decreased ATP -\> rigid RBCs
Increased 2,3 BPG -\> decr Hg affinity for O2
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Paroxysmal nocturnal hemoglobinuria
Increased complement destruction of RBC; impaired synthesis of GPI anchor for decay-accelerating factor
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Warm hemolytic anemia
IgG, seen in SLE and CLL
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Cold hemolytic anemia
IgM
CLL, mycoplasma, IM
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Acute intermittent porphyria
Decr Porphobilinogen deaminase; Porphobilinogen, ALA accumulate
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Porphyria cutanea tarda
Uroporphyrinogen decarboxylase deficiency
Uroporphyrin (tea- colored urine) builds up
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Iron poisoning
Cell death due to peroxidation of membrane lipids
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t(8;14)
Burkitt lymphoma (c-myc activation)
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t(9;22)
Philadelphia
(BCR-ABL hybrid)
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t(11;14)
Mantle cell lymphoma (cyclin D1 activation)
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t(14;18)
Follicular lymphoma (BCL-2 activation)
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t(15;17)
APL (M3 type of AML)
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Busulfan
Cross-links DNA
Treat CML
SE: myelosuppression, pulmonary fibrosis, hyperpigmentation
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Bevacizumab
MCA against VEGF; Inhibits angiogenesis
Treats Solid tumors
SE: Hemorrhage, blood clots, and impaired wound healing.
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Erlotinib
EGFR tyrosine kinase inhibitor.
Non-small cell lung carcinoma.
Rash.
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Cetuximab
Monoclonal antibody against EGFR.
Stage IV colorectal cancer (wild-type KRAS), head and neck cancer.
Rash, elevated LFTs, diarrhea.
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Imatinib
Tyrosine kinase inhibitor of BCR-ABL (Philadelphia chromosome fusion gene in CML) and c-kit (common in GI stromal tumors).
CML, GI stromal tumors (GIST)
Fluid retention.
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Rituximab
Monoclonal antibody against CD20, which is found on most B-cell neoplasms.
Non-Hodgkin lymphoma, CLL, ITP, rheumatoid arthritis.
Incr risk of progressive multifocal leukoencephalopathy.
54
Bortezomib, carfillzomib
Proteasome inhibitors, induce arrest at G2-M phase and apoptosis. Multiple myeloma, mantle cell lymphoma.
Peripheral neuropathy, herpes zoster reactivation.
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Tamoxifen, raloxifene
Selective estrogen receptor modulators (SERMs)—r- antagonists in breast and agonists in bone: Block binding of estrogen to ER ⊕ cells.
Breast cancer (tamoxifen only) and prevention
Raloxifene prevents osteoporosis.
Tamoxifen—partial agonist in endometrium: incr risk of endometrial cancer; “hot ashes.” Raloxifene—no risk of endometrial carcinoma (so you can relax!):estrogen receptor antagonist in endometrial tissue.
Both risk of thromboembolic events (eg, DVT, PE).
56
Trastuzumab (Herceptin)
Monoclonal antibody against HER-2 (c-erbB2), a tyrosine kinase receptor: inhibition of HER2-initiated cellular signaling and antibody- dependent cytotoxicity.
HER-2 ⊕ breast cancer and gastric cancer (tras2zumab). Cardiotoxicity.
“Heartceptin” damages the heart.
57
Vemurafenib
Small molecule inhibitor of BRAF oncogene ⊕ melanoma. VEmuRAF-enib is for V600E- mutated BRAF inhibition.
Metastatic melanoma.
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Rasburicase
Recombinant uricase that catalyzes metabolism of uric acid to allantoin
Prevention and treatment of tumor lysis syndrome.
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