Pathology: Hematopathology: Myelodysplasia/myeloproliferative Disorders, Block I

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Bone marrow stem cell disorders characterized by ineffective hematopoiesis and peripheral blood cytopenias

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Myelodysplastic Syndromes (MDS)

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This disease is characterized by patients greater than 50, blood cytopenias (often pancytopenia), worsening cytopenia due to progressive bone marrow failure, and possible progression to AML (1/3)

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Myelodysplastic Syndromes (MDS)

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What are the contributing factors in primary and secondary MDS?

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Primary- environmental exposure to agents may affect hematopoietic stem cells
Secondary- cytotoxic effects of chemotherapy

Front 4

This disease is characterized by increased myeloblasts, hyper or normocellular bone marrow, increased iron stores, and marrow fibrosis.

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MDS

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In this disease the myeloblasts in the bone marrow are less than 20%

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MDS

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These bone marrow stem cell disorders involve ineffective and disorderly hematopoiesis and may involve bone marrow failure - "preleukemia"

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Myelodysplastic Syndromes

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Hypercellular bone marrow with peripheral cytopenia

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Myelodysplasia

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Hypercellular bone marrow with peripheral hypercellularity

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Myeloproliferative

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The more myeloblasts in the bone marrow or peripheral blood, the greater the risk to pregression to ________.

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AML

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Bone marrow stem cell disorders resulting in disorderly and ineffective hematopoiesis (blood production) manifested by irreversible quantitative and qualitative defects in hematopoietic (blood-forming) cells. In a majority of cases, the course of disease is chronic with gradually worsening cytopenias due to progressive bone marrow failure. Approximately one-third of patients progress to AML within months to a few years.

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Myelodysplastic Syndrome

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Name three chromosome abnormalities in MDS

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1. Complete or partial loss of 5 or 7 (monosomy 5, monosomy 7, deletions of 5q or 7q)
2. Trisomy 8
3. Deletion of 20q

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The subtypes of MDS are classified based on what?

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The number of myeloblasts in the bone marrow - Must be less than 20%

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A clonal disorder arising from the neoplastic transformation of a multipotent bone marrow stem cell. Although only one cell line may have proliferative advantage, multiple marrow elements are derived from the abnormal stem cell.

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Multiproliferative Neoplasms (MPN)

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This disease originates in a pluripotent stem cell, and cells from granulocytic series have a proliferative advantage. Granulocyte pool may be 150x normal and peripheral blood contains large number of granulocytes in all stages of maturation.

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Chronic Myelogenous Leukemia

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Results from a balanced translocation of t(9;22) (q34;q11) - produces a chimeric bcr-abl gene which produces an mRNA with enhanced tyrosine kinase activity.

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Philidephia Chromosome

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The Philidelphia chromosome is not specific to this disease but is required for its diagnosis:

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CML (also seen in ALL)

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In the ________ phase of CML blasts account for 10-19% of WBCs

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Accelerated

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In blast crisis in CML blasts account for __% of WBCs in peripheral blood or bone marrow

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20% or more - clast crisis resembles acute leukemia

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In about 70% of CML cases, the blast crisis lineage is ______.

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Myeloid

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In 20-30% of cases, the blast phase of CML is due to proliferation of ____________.

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Lymphoblasts.

Front 21

This is the myeloproliferative disorder most strongly associated with activating point mutations in the tyrosine kinase JAK2.

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Polycythemia Vera

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Is a blood disorder in which the bone marrow makes too many red blood cells. It may also result in the overproduction of white blood cells and platelets. Most of the health concerns associated with this condition are caused by a blood-thickening effect that results from an overproduction of red blood cells

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Polycythemia vera

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What are the two phases of PV?

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1. Proliferative
2. Spent

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This disease has a phase characterized by an absolute increase in red cell mass with increased Hb/HCT. Hypercellular bone marrow with hyperplasia of all three cell lines.

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Polycythemia vera (proliferative phase)

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This condition has a phase characterized by marrow fibrosis with decreased cellularity, hepatospenomegaly, anemia, massive spenomegaly and bleeding.

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Polycythemia vera (Spent Phase)

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A classic symptom of this disease is itching, particularly after exposure to warm water (such as when taking a bath), which may be due to abnormal histamine release or prostaglandin production

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Polycythemia Vera

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A mutation in the JAK2 kinase was found to be strongly associated with this condition. JAK2 is a member of the Janus kinase family and makes the erythroid precursors hypersensitive to erythropoietin (EPO). This mutation may be helpful in making a diagnosis or as a target for future therapy.

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Polycythemia vera

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A myeloproliferative disorder characterized by sustained thrombocytosis with megakaryocytic hyperplasia in the bone marrow. JAK 2 is present in 50% of cases.

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Essential Thrombocythemia (ET)

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This is the least prevelent of the myeloproliferative disorders and presents in the 6th and 7th decades of life.

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Essential Thrombocythemia (ET)

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Is a rare chronic blood disorder characterized by the overproduction of platelets by megakaryocytes in the bone marrow in the absence of an alternative cause. In some cases this disorder may be progressive, and rarely may evolve into acute myeloid leukemia or myelofibrosis. It is one of four myeloproliferative disorders.

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Essential Thrombocythemia (ET)

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This disease is characterized by thrombosis, hemhorrhage, neurologic symptoms, and erythromelalgia (throbbing, aching or burning extremities caused by occlusion of the arterioles by platelet thrombi.

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Essential Thrombocythemia (ET)

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This disease is characterized by sustained thrombocytosis (600,000-1 million), morphologic heterogeneity of platelets (including giant platelets), mild anemia, normal wbcs, and hypercellular bone marrow with megakaryocytic hyperplasia.

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Essential Thrombocythemia (ET)

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Myeloproliferative disoreder characterized by obliterative marrow fibrosis, extramedullary hematopoesis, and often osterosclerosis. Also associated with the JAK2 protein.

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Primary Myelofibrosis

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In this disease, marrow fibrosis leads to distortion of the marrow sinusoids, permitting entry of immature hematopoietic cells that enter the peripheral blood and lodge in sites of extramedullary hematopoiesis - causes hepatospenomegaly and eventually portal hypertension.`

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Primary Myelofibrosis (PMF)

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This disease diagnosis includes splenomegaly, teardrop poikilocytes, increase in bone marrow reticulin fibers or collagen fibrosis.

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Primary Myelofibrosis (PMF)

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The peripheral blood in this condition has leukoerythroblastosis; presence of nucleated RBCs, teardrop RBCs, and immature myeloid cells to include myelocytes and metamyelocytes.

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Primary Myelofibrosis (PMF)