Term
| LAP activity in CML patients |
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Definition
| decreased LAP activity because of myeloid dysplasia |
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Term
| Major lineages involved in CML |
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Definition
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|
Term
| Major lineages involved in PV |
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Definition
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Term
| Major lineages involved in ET |
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Definition
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|
Term
| Major lineages involved in CIMF |
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Definition
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Term
| Abnormal complete blood count that suggests an MPD |
|
Definition
| erythrocytosis, granulocytosis, and/or thrombocytosis, dominance of transformed clone |
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Term
| Diagnostic chromosome abnormality associated with CML |
|
Definition
| Philadelphia chromosome, 9:22 translocation |
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Term
| Significance of chromosome abnormality associated with CML in ALL |
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Definition
| Philadelphia chromosome seen in 2-5% of childhood ALL, and 25% of adult ALL, identical to CML in blast crisis, the breakpoint on chromosome 22 differs and creates p190k protein |
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Term
| Peak incidence of CML according to age |
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Definition
| median age 60-80 years, rarely seen in childhood |
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Term
| Peak incidence of CML according to sex distribution |
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Definition
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|
Term
| Important peripheral blood findings in CML |
|
Definition
| increased leukocytes, increased immature granulocytes, decreased LAP |
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|
Term
| Important peripheral blood finding in CIMF |
|
Definition
| increased leukocytes, increased immature granuolcytes, decreased hemotocit |
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Term
| Important clinical findings in CIMF |
|
Definition
| increase in spleen size, and increase in bone marrow fibrosis |
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Term
| Important peripheral blood finding in PV |
|
Definition
| increase in hemocrit and increased platelets |
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|
Term
| Important peripheral blood finding in ET |
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Definition
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|
Term
| Laboratory findings typically associated with CIMF |
|
Definition
| peripheral blood has a leukoerythroblastic blood picture shows immature leukocytes and nRBCs also striking aniso and poik with dacryocytes, normochromic normocytic anemia with a WBC count <30,000/mL, leukocytosis or leukocytopenia, basophilia, eosinophilia, and pseudo-Pelger Huët, thrombocytosis or thrombocytopenia, micromegakaryocytes, megakaryocytes fragments |
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Term
| Peripheral blood findings in CML patients |
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Definition
| nRBCs, increased platelets, leukocytosis with left shift, predominant cells are segs and myelocytes, pros and blasts < 20%, increase in eosinophils and basophils, monocytes moderately increased, myeloid dysplasia leads to pseudo Pelger-huet and decreased LAP, occasionally MK fragments and micromegakaryocytes |
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|
Term
|
Definition
| BM aspiration yield dry tap, variable fibrosis, focal aggregates of megakaryocytes, fibrosis |
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Term
| Criteria that indicate a transformation of an MPD into a blast crisis |
|
Definition
| moderate to marked diffuse BM fibrosis, > 20% blasts in BM or BM, nRBCs in peripheral blood, extramedullary blast proliferation, presence of large clusters of blasts in the bone marrow biopsy specimen |
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|
Term
| Lab findings in primary PV |
|
Definition
| absolute increase in circulating RBC mass, thrombocytosis, and leukocytosis |
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|
Term
| Lab findings in secondary PV |
|
Definition
| appropriate and inappropriate increase in EPO |
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|
Term
| Characteristic peripheral blood picture in ET |
|
Definition
| extreme and consistent thrombocytosis, absence of increased RBC mass, normocytic normochromic, nRBCs, leukocytosis (22-40 x 10^3/ uL) |
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|
Term
|
Definition
| erythrocytosis, granulocytosis and/or thrombocytosis |
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|
Term
|
Definition
| panhypercellularity, panmyelosis, fibrotic |
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|
Term
| MPD other diagnostic laboratory test |
|
Definition
| cytogenetic abnormalities |
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|
Term
| Laboratory findings associated with MPD |
|
Definition
| erythrocytosis, granulocytosis, and/or thrombocytosis |
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|
Term
| Laboratory findings associated with AL |
|
Definition
| clonal expansion of a single tranformed cell and a decrease in normal cells |
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|
Term
|
Definition
| HSC is the cell or origin, balanced reciprocal translocation, juxtaposition of ABL proto-oncogene with portion of BCR gene |
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Term
| How Philadelphia chromosome leads to MPD and blast crisis |
|
Definition
| leads to production of BCR/ABL protein |
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|
Term
|
Definition
| novel BCR/ABL fusion gene produces novel 210-kD (p210) BCR/ABL fusion protein |
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|
Term
| How BCR/ABL1 rearrangement leads to MPD and blast crisis |
|
Definition
| abnormal tyrosine kinase activity, activates signal transduction pathways, increase in proliferation and decrease in apoptosis |
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|
Term
|
Definition
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|
Term
| How JAK2 (V617F) mutation leads to MPD and blast crisis |
|
Definition
| binds cytoplasmic domain of EPO-R |
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|
Term
| Expression of growth factor receptors |
|
Definition
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|
Term
| How expression of growth factor receptors leads to MPD and blast crisis |
|
Definition
| receptor activation independent of EPO binding and signaling, increase in Bcl-X leads to decrease in apoptosis of progenitor cells |
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|
Term
|
Definition
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|
Term
| Benign leukemoid reaction |
|
Definition
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|
Term
| Role of growth factors in fibrosis from CIMF |
|
Definition
| mutation of MPL- (W515L) which is a TPO membrane receptor, due to fibrinogen GFs, stimulate growth of and proliferation of fibroblasts and accumulation of BM stromal elements |
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|
Term
| Laboratory results peripheral blood in CIMF |
|
Definition
| immature leukocytes and nRBCs, striking aniso and poik, normochromic and normocytic anemia, WBCs < 30,000/mL, leukocytosis and leukocytopenia, basophilia, eosinophilia, and pseudo- Pelger Huët, thrombocytosis or thrombocytopenia, micromegakaryocytes and megakaryocyte fragments |
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|
Term
| Laboratory results bone marrow in CIMF |
|
Definition
| dry tap, variable fibrosis, focal aggregates of megakaryocytes |
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|
Term
| Relative PV laboratory findings |
|
Definition
| decrease in plasma volume due to dehydration |
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|
Term
| Absolute PV laboratory findings |
|
Definition
| increase in RBC in peripheral blood, other progeny of HSC may be increased and erythrocytosis leads to increased hemoglobin, hematocrit and RCM |
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|
Term
| Essential thrombocytopenia laboratory results |
|
Definition
| increase in MK proliferation, thrombocytopathy, qualitative platelet disorder, extreme and consists thrombocytosis, >600 x 103/uL, bizarre platelets, megakaryocytes and fragments, anemia, leukocytosis |
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|
Term
| Non-malignant conditions the result in thrombocytosis |
|
Definition
| Chronic inflammatory disorders, acute hemorrhage, postsplenectomy, postoperative, and iron deficiency |
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