Leukemias and Myelodysplastic Syndromes Flashcards
Give 3 similarities between myeloproliferative neoplasms and myelodysplastic syndrome.
They are both quantitative, clonal stem cell disorders, and can transform into acute leukemia
Give 3 differences between myeloproliferative neoplasms and myelodysplastic syndrome.
Myeloproliferative have cytosis, normal cell morphology, and organomegaly.
Myelodysplastic have cytopenia/ineffective hematopoeisis, abnormal cell morphology, and minimal organomegaly
What’s the difference between leukemia and lymphoma?
Leukemia occurs primarily in bone marrow; lymphoma involves extramedullary tissues, esp lymph nodes
Give the 8 types of myeloproliferative neoplasms according to WHO.
Bcr-abl1 positive CML, polycythemia vera, primary myelofibrosis, essential thrombocythemia, chronic neutrophilic leukemia, chronic eosinophilic leukemia, mastocytosis, myeloproliferative neoplasm unclassifiable
Which myeloproliferative neoplasm is defined primarily by granulocyte proliferation over the full range of myeloid maturation?
CML (main granulocyte = neutrophil)
Which myeloproliferative neoplasm is defined by erythrocyte, megakaryocyte, and granulocyte proliferation?
Polycythemia vera
Which myeloproliferative neoplasm is defined by proliferation of megakaryocytes and granulocytes, but not erythrocytes?
Primary myelofibrosis
Which myeloproliferative neoplasm is defined by megakaryote proliferation only?
Essential Thrombocythemia
Which myeloproliferative neoplasm is defined by the uncontrolled proliferation of mast cells only?
Mastocytosis
In which myeloproliferative neoplasm does marrow fibrosis occur most frequently? What cells are responsible for stimulating the fibroblasts?
Primary myelofibrosis; megakaryotes stimulate fibroblasts
What kind of shift occurs when the number of younger, less well differentiated neutrophils and neutrophil-precursor cells in the blood increases? This generally reflects early or premature release of myeloid cells from the bone marrow.
Left shift
Philadelphia chromosome / bcr-abl / t(9;22) is associated with what disease? What size protein is typically made and what enzyme function does it have?
CML; 210kd tyrosine kinase
Polycythemia vera, primary myelofibrosis, and essential thrombocytopenia all tend to have mutations that activate what protein? How does this contribute to disease progression?
JAK2 gain-of-function: cells become hypersensitive to growth factors and cytokines
Systemic mastocytosis generally involves an activation of what proto-oncogene?
C-kit
In which of the following neoplasms does secondary or refractory cytopenia occur: Bcr-abl1 positive CML, polycythemia vera, primary myelofibrosis, essential thrombocythemia, and mastocytosis, MDS, AML?
Polycythemia vera, primary myelofibrosis, and mastocytosis, MDS, and AML (the first 3 end up with hypocellular bone marrow due to fibrosis; MDS is due to ineffective release from marrow; AML is due to accumulation of immature myeloid cells in the marrow)
