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    Aplastic Anemia

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    musab
    Aplastic anemia is a bone marrow failure syndrome caused by stem cell injury or dysfunction. It can be congenital, idiopathic, or caused by toxins, drugs, infections, or autoimmune diseases. Patients present with pancytopenia and are at risk for infection and bleeding. Treatment depends on severity but may include blood transfusions, immunosuppressive therapy, bone marrow transplant, or androgens. Prognosis is rapidly fatal without treatment but bone marrow transplant can result in durable response in 80% of cases. Pure red cell aplasia is a rare autoimmune cause of isolated anemia where red blood cell precursors are damaged. It can be idiopathic or associated

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    Aplastic Anemia

    Uploaded by

    musab
    25 views19 pages
    Aplastic anemia is a bone marrow failure syndrome caused by stem cell injury or dysfunction. It can be congenital, idiopathic, or caused by toxins, drugs, infections, or autoimmune diseases. Patients present with pancytopenia and are at risk for infection and bleeding. Treatment depends on severity but may include blood transfusions, immunosuppressive therapy, bone marrow transplant, or androgens. Prognosis is rapidly fatal without treatment but bone marrow transplant can result in durable response in 80% of cases. Pure red cell aplasia is a rare autoimmune cause of isolated anemia where red blood cell precursors are damaged. It can be idiopathic or associated

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    Aplastic anemia

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    Aplastic anemia is a bone marrow failure syndrome caused by stem cell injury or dysfunction. It can be congenital, idiopathic, or caused by toxins, drugs, infections, or autoimmune diseases. Patients present with pancytopenia and are at risk for infection and bleeding. Treatment depends on severity but may include blood transfusions, immunosuppressive therapy, bone marrow transplant, or androgens. Prognosis is rapidly fatal without treatment but bone marrow transplant can result in durable response in 80% of cases. Pure red cell aplasia is a rare autoimmune cause of isolated anemia where red blood cell precursors are damaged. It can be idiopathic or associated

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    25 views19 pages

    Aplastic Anemia

    Uploaded by

    musab
    Aplastic anemia is a bone marrow failure syndrome caused by stem cell injury or dysfunction. It can be congenital, idiopathic, or caused by toxins, drugs, infections, or autoimmune diseases. Patients present with pancytopenia and are at risk for infection and bleeding. Treatment depends on severity but may include blood transfusions, immunosuppressive therapy, bone marrow transplant, or androgens. Prognosis is rapidly fatal without treatment but bone marrow transplant can result in durable response in 80% of cases. Pure red cell aplasia is a rare autoimmune cause of isolated anemia where red blood cell precursors are damaged. It can be idiopathic or associated

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    Aplastic anemia

    - BM failure that arises from injury to or abnormal


    expression of the stem cell (hypoplastic BM/ pancytopenia)
    - Causes: - congenital
    - idiopathic (?autoimmune)
    - SLE
    - chemotherapy/radiotherapy
    - toxins: benzenes, tolune, insecticides
    - drugs: CAF, phenylbutazone, Au, sulfa
    - post- hepatitis
    - pregnancy
    - PNH
    Clinical features
    • Anemia (severe: neutrophil <500/mcL
    platelets <20,000
    BM cellularity <20%
    retic count <1%
    • vulnerability to bacterial infection
    • mucosal/skin bleeding
    • pallor, purpura, petechie
    Pancytopenia, causes
     BM disorder
     Aplastic anemia
     MDS
     acute leukemia
     MF
     infiltrative diseases
    (lymphoma, myeloma, Ca)
     megaloblastic anemia
     nonmarrow disorders
     hypersplenism
     SLE
     infections (TB, AIDS, leishmaniasis, brucellosis)
    Treatment
    supportive (most)
    • transfusion (RBC, platelets)
    • antibiotics
    in severe cases + young <50: allogenic BMT
    (HLA-matched)
    elderly (>50): - immunosuppressive + anti-thymocyte
    globulin (ATG) + cyclosporin (or tacrolimus)
    high dose immunosuppressive with
    cyclophosphamide, 200mg/kg
    androgens (oxymetholone) 2-3mg/d
    Course & prognosis
    rapidly fatal if not treated
    ABMT: durable complete response 80%
    ATG Rx: partial response 60%
    long-term responders: good prognosis
    Pure red cell aplasia
    -Rare
    - autoimmune disease against erythroid precursors
    - idiopathic
    - SLE, CLL, lymphoma, thymoma
    - drugs: phenytoin, CAF)
    - in response to viral infection- papovirus
    - anemia: often severe, NCNC
    - low/absent reticulocytes
    - RBC morphology : N
    - myeloid & platelet lines not affected
    - BM: normocellular, erythroid precursors are markedly reduced or absent
    - treatment:
    stop offending drug
    resection of the tumor( eg, thymoma)
    high –dose iv Ig (eg, papovirus – related)
    immunosuppressive therapy: ATG+ cyclosporine (tacrolimus)
    anti-CD20 monoclonal antibody (rituximab)
    Myeloproliferative Disorders
    -Due to acquired clonal abnormality of the
    hematopoeitic stem cells
    Classification:
    - myeloproliferative syndromes
    • PV
    • MF
    • ET
    • CML
    - MDS
    - AML
    PV
    • overproduction of all the 3 hematopoeitic cells lines (most RBC)
    • autonomous production of erythroid cell in the BM
    • independent of EPO
    • mutation in JAK2 gene, 95%
    Clinical
    - Sxs related to expanded plasma volume & increased blood
    viscosity
    - headache, dizziness, tinnitus, blurred vision, fatigue
    - generalized pruritus
    - epistaxis
    - male predominance (60%)
    - median age: 60yrs
    - plethora & engorged retinal vessels,
    - spleen always enlarged (75% palpable)
    - thrombosis is the commonest complication
    - high incidence of PUD
    Lab
    - Hct >60%
    - WBC 10,000-20,000/mcL
    - platelets > 1,000,000/mcL
    - basophilia + eosinophilia
    - BM: hypercellular
    panhyperplasia
    absent Fe store
    - Vit B12
    - hyperurecemia
    Treatment
    - Phlebotomy
    - 1 unit/week
    - target : Hct <45%
    - myelosuppressive therapy
    - indication: - high phlebotomy requirement
    - thrombocytopenia
    - intractable pruritus
    - HU 500-1500mg/d
    - anaglinide
    - aspirin (75-81mg)
    - allopurinol
    - antihistamines
    - prognosis
    - survival : 11-15 years
    - may arrest MF/CML
    - AML (5%)
    ET
    - Uncommon
    - unknown cause
    - marked proliferation of megakaryocytes in BM
    leading to elevation of the platelet count
    - mutation of JAK2 (50%)
    Clinical
    -Median age of presentation: 50-60 yrs
    - increased platelet count
    - thrombosis most common
    - erythromelalgia
    - splenomegaly 25%
    Lab
    - Platelet 2,000,000/mcL
    - WBC <30,000/mcL + immature myeloid forms
    - Hct: N
    - BF: large platelets
    - BM: megacaryocytes
    Treatment
    -HU 0.5-2g/d
    - anaglide 1-2mg/d
    - aspirin
    - plateletphresis
    Course & prognosis
    - Indolent
    - average survival: >15yrs
    - late course fibrotic BM + massive splenomegaly
    - 10-15% risk of progression to MF after 15 yrs, 1-5% to AML over
    20 yrs
    ET- BM
    MF
    - In adults >50
    - usually insidious in onset
    - present with fatigue (anemia), abdominal fullness
    (splenomegally)
    - unusual: bleeding, bone pain
    - splenomegally invariable & massive
    - heptomegaly 50%
    - anemia + progressive thrombocytopenia
    bleeding
    - hematopoeisis in the liver portal hypertension,
    ascites, esophageal varices
    Lab
    - Anemia
    - WBC variable
    - platelet variable
    - peripheral blood
    - significant poikilocytosis
    - tear drop forms
    - nucleated RBC
    - myeloid features shifted to immature forms
    - platelet morphology bizzare (giant degranulated form)
    - megakaryocyte fragments
    - BM: - dry tap
    - fibrosis, reticulum fibers
    collagen
    - No specific chromosomal
    abnormality
    Treatment
    -Mild form: no Rx
    - transfusion
    - thalidomide / lenalidomide
    - allogenic BMT
    - EPO
    - splenectomy
    Course & prognosis
    -Median survival from the time of Dx: 5yrs
    - end-stage MF
    -Generalized debility
    - liver failure
    - bleeding (thrombocytosis)
    - some terminate with AML
    MDS
    - A group of acquired clinical disorders of
    hematopoeitic stem cells
    - xed by cytopenias, hypercellular BM, a number of
    morphologic & cytogenic abnormalities
    - usually idiopathic
    - may be after cytotoxic chemotherapy
    - no specific chromosomal abnormality
    - contains heterogenous syndromes
     without excess BM blasts = refractory anemia with or
    without ringed sideroblasts
     with excess blasts (RAEB 5-19% blasts)
     with proliferative syndrome = CMML (monocytosis)
    >1000/mcL
    Clinical
    -Usually > 60 yrs
    - 1st diagnosed because of abnormal counts
    - fatigue, infection, bleeding
    - course indolent
    - wasting illness (fever, wt, loss, general debility)
    - splenomegally may be present
    Lab
    - Marked anemia
    - MCV: N / increased
    - macro-ovalocytes
    - WBC: N/ decresed
    - neutropenia common
    - deficient granules or bilobed nucleus (Pelger-Huet)
    - myeloid series: left shifted
    - platelet : N/decreased; hypogranular platelet
    -BM: - xically hypercellular
    - erythroid hyperplasia
    - sign of abnormal erythropoisis
    - megaloblastic features
    - nuclear budding
    - multinucleated erythroid precursors
    - ringed siderblasts
    - dwarf megakaryocyte with a unilobed nucleus
    - variety of cytogenic abnormality (5q- syndrome, monosomy 7)

    - treatment
    - blood transfusions
    - EPO, 30,000 U ,sc
    - myeloid granulocyte factors (G-CSF) + EPO
    - lenalidomide, 10mg/d
    - azacidine
    - decitabine
    - immunosuppressive (anti-thymocyte globulin)
    - allogenic stem cell transfusion - curative
    Course & prognosis
    - Ultimately fatal
    - cure rate: 30-60%
    - most die of infection/ bleeding
    - pts with refractory anemia may survive many yrs
    (risk of leukemia: <10%)
    - Those with excess blasts or CMML: short survival
    (<2 yrs) & higher (20-50%) risk of developing
    acute lekemia
    - full deletion of C5 and C7: poor prognosis

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