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15 Cards in this Set
- Front
- Back
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What are some causes of pancytopenia (what's on your differential)? KNOW
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Aplastic anemia
Myelodysplastic syndromes (MDSs) Bone marrow infiltration (leukemia, lymphoma, carcinoma, granulomatous disease, fibrosis, congenital storage diseases Nutritional deficiencies (B12/folate) Bone marrow toxins (chemo, radiation, chemicals, alcohol) Infections (HIV, EBV, parvovirus, hepatitis, histoplasmosis, tuberculosis) Hypersplenism Systemic disorders (SLE) Anorexia nervosa |
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What is the definition of aplastic anemia?
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Pancytopenia + hypocellular bone marrow
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What is the clinical presentation of aplastic anemia?
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Anemia: pallor, fatigue, chest pain, SOB, headache
Neutropenia: fever, infections Thrombocytopenia: bruising, petechiae, epistaxis, gingival bleeding, menorrhagia |
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How do you diagnose aplastic anemia?
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Blood counts
Reticulocyte count BM biopsy Rule out other causes |
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What is the pathogenesis of aplastic anemia?
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Damage/destruction/suppression of hematopoietic stem cells leads to depletion of stem cell pool
Generally either direct toxic effect to stem cells or immune-mediated suppression of stem cells |
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How do you treat aplastic anemia (severe or very severe)?
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Age < 20: hematopoietic stem cell transplant (HSCT) if donor available or immunosuppression
Age 20-45: HSCT if healthy and donor available or immunosuppression Age > 45: Immunosuppression |
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What are some features of immunosuppression for aplastic anemia?
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Antithymocyte globulin + cyclosporin
About 2/3 response rate, but takes 3-4 months to see response They can relapse, but respond to the same treatment again |
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What is myelodysplastic syndrome?
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Clonal proliferation of hematopoietic stem cell with dysplasia
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How does MDS present?
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Cytopenia (symptoms below relate to this)
Fatigue, dyspnea, angina, headache (anemia) Neutropenic infections hemorrhage, bruising (thrombocytopenia) Organomegaly Bone/joint pains Weight loss Night sweats |
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What does the peripheral blood smear of MDS look like?
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Dyserythropoiesis (RBC anisopoikilocytosis)
Dysgranulopoiesis (hypogranular neutrophils) Dysmegakaryopoiesis (multiple separate nuclei and unilobate megakaryocytes) |
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What is a subtype of MDS that has a poor prognosis?
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MDS resulting from alkylating agents or radiation exposure
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What is chronic myelomonocytic leukemia?
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A dysplasia in at least one lineage in the bone marrow but with <20% blasts with absolute monocytosis in the peripheral blood
BCR/ABL negative Important to check for 5q31-33 because these patients respond well to imatinib |
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What is the 5q- syndrome?
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Mainly females around 70
Isolated macrocytic anemia with normal/elevated platelets but bone marrow erythroid hyperplasia Very good prognosis, that's why it's important to detect |
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What are the main goals in MDS treatment?
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Control cytopenic symptoms
Improve quality of life Improve overall survival Decrease progression to AML |
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What are the treatment options for MDS? Give a little blurb about each.
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Observation
Allogeneic HSCT (only curative treatment and realistic only for a subset; high risk) Intensive chemotherapy (never do this, doesn't work) Supportive care/hematopoietic growth factors (mainstay of initial therapy for low/medium risk MDS patients; recombinant human EPO, especially good for those with low EPO levels and low transfusion requirements; also chelation therapy to prevent iron overload from multiple transfusions, but probably isn't going to do much) Immunosuppression (in a subset of patients, the problem is clonal amplification of T-lymphocytes; treatment is antithymocyte + cyclosporine; works better in less severe cases) DNA methyltransferase inhibitors (unroll chromatin for protector-genes activation; takes 3-4 months before you can tell if it's working) Immunomodulatory agents (lenalidomide; anti-apoptosis agents, anti-angiogenesis) Participation in a clinical trial |
