Myeloproliferative Dx Flashcards
In myeloproliferative disorders each disorder is caused by what?
clonal expansion that arises from a pluripotent stem cell
What are the 4 conditions associated with Myeloproliferative Disorders
- Polycythemia vera (RBCs)
- Essential (1o) Thrombocythemia (platelets)
- Chronic Myelogenous Leukemia (granulocytes)
- Myelofibrosis (collagen or fibrous tissue)
Which type of disorder group has the following results: Abnormal, excessive production of erythroid, myeloid, and megakaryocytic precursors in bone marrow?
Myeloproliferative Disorders
Myeloproliferative disorders are reflected in which 2 labs?
peripheral blood smear & CBC results
Myeloproliferative disorders are often caught on a CBC with what or by what on physical exam
irregular cell counts; splenomegaly
What disorder group is indicative of the following presenting symptoms include: – Potentially None – Hypertension – Itching – Burning feet – Bleeding – Thrombosis – An enlarged spleen (leading to abdominal fullness and discomfort)
Myeloproliferative Disorders
What is a chronic myeloproliferative disorder characterized by increased Hgb concentration & increased RBC mass?
Polycythemia Vera (PV)
Which condition has the following characteristics?
- Mean age at diagnosis is 60 years old
- Environment: exposure to high levels of radiation may increase risk
- CBC and peripheral smear show panmyelosis: increased RBCs, WBCs, & Platelets
Polycythemia Vera (PV)
Which class of polycythemia is due to low plasma volume: dehydration, burns, diuretics?
Relative
Which class of polycythemia has the following characteristics?Hypoxia, seen with smoking, high altitude, lung disease
Secondary “Reactive Polycythemia”
Which class of polycythemia has the following characteristics? “Malignant” hematologic disorder, not self-limiting. Treated with phlebotomy, anti-neoplastic drugs. Significant increases in all formed elements
Primary Polycythemia Vera
Which myeloproliferative disorder has the following signs and symptoms?
• Expanded blood volume & hyperviscosity lead to: weakness, headache, light-headedness, visual disturbances, fatigue, dyspnea
• Epistaxis
• Pruritus is frequent
• Face may be red, retinal veins engorged
• Hypertension
• Hepatomegaly frequent
• Splenomegaly in > 75% of patients
Polycythemia vera
In polycythemia, oxygen content of blood _________, but oxygen saturation of tissues is ________.
increased; decreased
In polycythemia, tissue hypoxia due to what?
increased blood viscosity, causing problems with tissue perfusion & vessel thrombosis
In polycythemia, which phenomenon is common?
Raynaud’s
Chronic cases of polycythemia results in increased ______ ______ & increased ______ _______ in an effort to decrease tissue hypoxia
cardiac output; capillary beds
Which Myeloproliferative disorder has the following peripheral blood findings?
• Immature WBCs • Immature RBCs, with marked anisocytosis and poikilocytosis: microcytes, elliptocytes, and dacrocytes may be seen • Neutrophilia with abnormal morphology • Thrombocytosis with abnormal morphology & if function is also abnormal then increased bleeding
Polycythemia vera
Which myeloproliferative disorder has an idiopathic platelet count increase above 500,000/µL, in the absence of the features of the other myeloproliferative disorders and has the following characteristics?
• Seen in 50-70 year olds
• Characterized by:
• Markedly increased platelet count
• Bone morrow megakaryocyte hyperplasia
• Either a hemorrhagic or thrombotic tendency.
Essential (1o) Thrombocythemia
Which myeloproliferative disorder has the following diagnostic conditions?
• Platelets 500,000 to 1,000,000
• Normal RBC mass
• Lack of BM fibrosis
• Absence of dacrocytes
• Absence of “Philadelphia Chromosome” (CML)
Essential (1o) Thrombocythemia
Which myeloproliferative disorder is a condition in which bone marrow becomes fibrotic and is characterized by:
- BM fibrosis
- Splenomegaly
- Normo/normochromic anemia with Dacrocytes
- Myeloid metaplasia: Extramedullary hematopoiesis, cells formed in liver & spleen
Myelofibrosis
Which myeloproliferative disorder has the following characteristics?
• Peak incidence 50-70 years
• Median survival 10 years from onset
• Dx by BM biopsy showing fibrosis, marrow aspiration commonly dry • WBC & platelet counts frequently high initially, become low as disease progresses
Myelofibrosis
Which myeloproliferative disorder has the following CBC results?
• Leukoerythroblastic: Peripheral blood picture immature WBCs and RBCs.
• RBCs: Normocytic normochromic anemia mild poikilocytosis, polychromatophilia & NRBCs, Dacrocytes
• WBCs: Initial leukocytosis with immature neutrophils
• Platelets: eventually thrombocytopenia
Myelofibrosis
The following are other conditions that may manifest as what?
- PV: 15-30% of cases
- Leukemias, lymphomas, multiple myeloma
- TB and osteomyelitis
- Myelodysplastic Syndrome MDS
- Exposure to benzene, X-Rays, Gamma Rays
a “secondary myelofibrosis” as part of their respective disease courses
What are the following DDx conditions characteristic of?
- Acute infection
- Chronic inflammatory dz (RA, TB)
- Iron deficiency anemia
- Hemolysis
- Cancer
- Lymphoma
- Splenectomy
DDx from causes of 2o Thrombocythemia
Which myeloproliferative disease is the analogy of the kid that never leaves home and lives in their parent’s basement?
Myelodysplasia (MDS)
Which myelodysplasic syndrome is a heterogenous group of disorders that result in ineffective hematopoiesis, and has the following characteristics?
- USUALLY ALL 3 cell lines involved.
1. Proliferation of abnormal clones of hematopoietic cells in BM, but with ineffective & abnormal blood cell production leading to cytopenias, with extramedullary hematapoiesis in liver and spleen resulting in splenomegaly and hepatomegaly.
2. BM may be normal or hypercellular & contains
MDS