Leukemias and Myeloid Disoders 2 Flashcards
Myeloproliferative diseases and syndromes
Clonal productive neoplastic proliferations with cell maturation
Marrow hyercellularity and splenomegaly
Elevated blood counts
JAK2 mutations often
POlycythemia vera
Elevation in RBC mass increased platelet and granulocyte counts and basophilia
More males
POlycythemia vera symptoms
Hypertension and poor platelet function
Enlargement of organs
Gradual transition to spent phase
POlycythemia veria causes
JAK2 kinase
MUST have low EPO to distinguish from secondary polycythemia or relative polycythemia
Increased uric acid and histamine release
Chronic ideopathic myelofibrosis
Marrow fibrosis related to PDGF and TGFbeta from abnormal megakaryocytes
Edxtramedulary heamtopoiesis with splenomegaly
Anemia with teardrops and leukoerythroblastic picture
JAK2 in 50-60%
Essential thrombocytopneia
Sustained elevated platelet count
Thrombosis and hemorrhage probs
JAK2 50-60%
Myelodysplastic syndromes
Usually refractory cytopenias which may precede AML
Changesi nbone marrow
Must have more than 20% blasts in bone marrow
Adults over 50
Pancytopenia
Rule out vitamin deficiency
Frequent cytogenic abnormalilty (5,7,8)
Myelodysplastic/myeloproliferative dzs
Effective porliferation of one cell line with cytopenias of other
CMML - chronic myelomonocytic leukemia - overlap with monocytosis
CLL/SLL age and what to look for in blood counts nad morphology
Over 50 - most common in adults in western hemisphere
B cell disorder with CD19, dim CD20, CD5, and CD23 co-expression and light chain restirction (all kappa or lmbda)
More than 000 peripheral lymphocytes, SLL if nodal presnetation and WBC not increased
Small mature lymphos with clumped chromatin
Symptoms of CLL
LAD, hepatosplenomegaly IMmune dysfunction (positive coombs 15%, hypogammaglobulinemia 50%)
Hairy cell leukemia
Chronic clonal mature B cell disorder MUCH more in men Fried egg Pancytopenia and monocytopenia Infections common Splenomegaly
Hairy cell leukemia immunophenotype
CD19, 20, right CD22, andf 11c, CD103, and CD25
Hair cell prognosis
Drug therapy effective
2-CDA and pentostatin
Neutropenia causes
Aplastic anemia Bone marrow replacement Drugs Ineffective hematoposiesis (vit def) Accerleared removal Serious infections when count low
Lymphopenia causes
Less common (HIV)