Term
| Sequence of granulocyte differentiation? (5) |
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Definition
Promyelocyte
Myelocyte
Metamyelocyte
Band
Granulocyte (Neutrophil, Eosinophil, Basophil) |
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Term
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Definition
All glucose, anaerobic
90% via lactate dehydrogenase
10% via HMP shunt |
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Term
Platelets:
Dense granules?
Alpha granules?
vWF receptor?
Fibrinogen receptor?
Life span? |
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Definition
Dense: ADP, calcium
Alpha: vWF, fibrinogen
vWF: GpIb
Fibrinogen: GpIIb/IIIa
Lifespan: 8-10 days |
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Term
| Mast cell granule contents? |
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Definition
Histamine
Heparin
Eosinophil chemotactic factors
Tryptase (specific marker) |
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Term
| Basophil granule contents? |
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Definition
Histamine
Heparin
Leukotrienes (D4) |
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Term
| Eosinophil granule contents? |
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Definition
Major basic protein (anti-helminthic)
Histaminase, arylsulfatase (anti-allergenic) |
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Term
| Neutrophil granule contents? |
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Definition
Hydrolytic enzymes
Lysozyme
Myeloperoxidase
Lactoferrin (multiple antibiotic effects) |
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Term
| Bernard-Soulier syndrome? |
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Definition
| Lack GpIb. Platelets cannot bind vWF. |
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Term
| Glanzmann's thrombasthenia? |
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Definition
| Lack GpIIb/IIIa - no fibrinogen crosslinking. |
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Term
| Hereditary sideroblastic anemia |
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Definition
X-linked defect in ALA synthase gene.
Ringed sideroblasts (RBC w/ iron laden mitochondria), high Fe, normal TIBC, high ferritin.
B6 therapy. |
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Term
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Definition
LEAD
Lead lines on gingiva (Burton's lines)
Encephalopathy and Erythrocyte basophilic stippling
Abdominal colic, sideroblastic Anemia
Drops: wrist and foot.
Dimercaprol, EDTA, Succimer in children |
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Term
| Anemia of chronic disease |
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Definition
Inflammation causes increased hepcidin release, decreased release of iron from macrophages.
Low Fe, low TIBC, high ferritin. |
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Term
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Definition
Radiation
Drugs (chemo, toxins)
Viral (B19, EBV, HIV, HCV)
Fanconi's anemia (congenital DNA repair defect)
Idiopathic (autoimmune, may follow acute hepatitis) |
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Term
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Definition
| mutation in heme binding pocket of alpha or beta chain. Most common His -> Tyr. Persistent methemoglobinemia (resists reduction to Fe2+) |
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Term
Give lab values: Fe, TIBC, Ferritin, %Transferrin saturation.
Fe deficiency
Chronic disease
Hemochromatosis
Pregnancy/OCPs
Lead poisoning |
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Definition
Fe def: low Fe, high TIBC, low ferritin, low T%
ChronicDz: low Fe, lower TIBC, high ferritin, high T%
Hemo: high Fe, low TIBC, high ferritin, high T%
Preg: norm Fe, high TIBC, norm ferritin, low T%
Lead: high Fe, low TIBC, norm ferritin, high T% |
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Term
| Acute intermittent porphyria |
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Definition
Lack porphobilinogen deaminase.
Accumulate porphobilinogen, ALA, uroporphyrin (in urine).
Sx: 5Ps
Painful abdomen
Pee red
Polyneuropathy
Psychological disturbance
Precipitated by drugs
Tx: glucose and heme, inhibit ALA synthase |
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Term
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Definition
Lack uroporphyrinogen decarboxylase.
Accumulate uroporphyrin - tea colored urine
Blistering cutaneous photosensitivity.
Most common porphyria. |
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Term
| Idiopathic Thrombocytopenic Purpura |
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Definition
anti-GpIIb/IIIa antibodies. Platelet destruction.
Increased megakaryocytes. |
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Term
| Thrombotic thrombocytopenic purpura |
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Definition
Antibodies to ADAMTS13 (vWF metalloprotease).
Large vWF multimers, platelet aggregation, thrombosis.
Schistocytes, high LDH (microangiopathic hemolytic anemia).
PENTAD: Neuro, Renal, Fever, Thrombocytopenia, Microangiopathic hemolytic anemia |
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Term
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Definition
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Term
| JAK2 mutations associated with what diseases? |
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Definition
Chronic myeloproliferative disorders
(Polycythemia vera, Essential thrombocytosis, Myelofibrosis)
Increasingly sensitive to growth factors. |
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Term
| What markers indicate better ALL prognosis? (2) |
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Definition
t(12;21)
CD10 (CALLA) positive |
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Term
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Definition
Absence of erythroid cells and precursors, with normal granulopoiesis, thrombopoeisis.
Idiopathic, thymoma association, or parvovirus B19 associated (preferentially destroys proerythroblasts). |
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