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Primary BM disorders (Primary myelofibrosis (Tx (Ruxolitinib, Transfusion,…
Primary BM disorders
Primary myelofibrosis
Proliferation hematopoietic cell clones (particularly megakaryocytes) --> releases cytokines like fibroblasts --> fibrosis, collagen produciton, osteosclerosis, CD34 cell mobilisation --> hematopoetic tissue replaced by connective tissue and fibrosis
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Dx
PS - tear drop cells, BM infiltration with blasts, nucleated RBCs, myelocytes - leukoerythroblastic picture
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CBC - low Hb, high WBC, high platelet, high LDH
Cytogenetics - JAK2, V617F, CALR mutation negative
Clinical px
Anemia symptoms - dyspnea, reduced ET
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Increase WBC, decrease Hb
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Myelodysplastic syndrome
Potential for hematological emergencies - can lead to microangiopathic hemolytic anemia due to anemia + thrombocytopenia
BM failure - thrombocytopenia, macrocytic anemia
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Classification
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RAEB-I - refractory anemia with excess blasts, 5-9%
RAEB-II - refractory anemia with excess blasts, 10-19%
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Immature blood cells don't mature - non-functioning BM; direct insult of hematopoietic stem cells leading to increased apoptosis or clonal mutation and expansion of an abnormal clone leading to ineffective hematopoiesis
Possible progression to AML due to aberrant growth signals, reduced apoptosis --> uncontrolled blast cell proliferation
Aplastic anemia
Congenital AA
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Growth retardation, congenital skeletal defects, renal defects, skin defects, mental retardation
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Tx - androgen, BM transplant
Acquired AA
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Tx - anti-thymocyte globulin, cyclosporin, methylprednisolone, BM transplant
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Dx
CBC - pancytopenia, severity depends on neutrophil count
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BM study - hypocellular, hypoplasia (empty bone marrow), mostly fat cells,very little functional cells, cellularity <25%
Causes - 5I
Iatrogenic - chemo drugs, radiation, cytotoxic drugs
Idiopathic
Most common type, may be immune related
Proliferation defect of precursor cells, stem cell defects or immune reaction against hemopoietic tissues
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Infections - EBV, HBV, HIV
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Inherited
Fanconi's anemia
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Progressive BM failure, increased malignancy predisposition
Growth retardation, somatic abnormalities
Cafe au lait spots, hypo/hyperpigmentation
Skeletal abnormalities - absent thumb, radial hypoplasia, scoliosis
Underdeveloped gonads, horseshoe kidneys
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Tx
Supportive
Transfusion - red cells, platelets
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