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    MYELOFIBROSIS Final Yr 2020

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    HEMA
    Myelofibrosis is a clonal hematologic malignancy characterized by the replacement of normal bone marrow with fibrous tissue. It can develop primarily (called primary myelofibrosis) or secondary to other hematologic malignancies or diseases. The main symptoms include an enlarged spleen, low blood cell counts, and constitutional symptoms. Diagnosis involves blood tests and a bone marrow biopsy showing fibrosis. Risk stratification guides treatment, which may include drugs, splenectomy, stem cell transplant depending on disease severity and patient fitness. JAK inhibitors like ruxolitinib are first-line for symptomatic patients not eligible for transplant.

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    MYELOFIBROSIS Final Yr 2020

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    HEMA
    18 views14 pages
    Myelofibrosis is a clonal hematologic malignancy characterized by the replacement of normal bone marrow with fibrous tissue. It can develop primarily (called primary myelofibrosis) or secondary to other hematologic malignancies or diseases. The main symptoms include an enlarged spleen, low blood cell counts, and constitutional symptoms. Diagnosis involves blood tests and a bone marrow biopsy showing fibrosis. Risk stratification guides treatment, which may include drugs, splenectomy, stem cell transplant depending on disease severity and patient fitness. JAK inhibitors like ruxolitinib are first-line for symptomatic patients not eligible for transplant.

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    MYELOFIBROSIS final yr 2020

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    Myelofibrosis is a clonal hematologic malignancy characterized by the replacement of normal bone marrow with fibrous tissue. It can develop primarily (called primary myelofibrosis) or secondary to other hematologic malignancies or diseases. The main symptoms include an enlarged spleen, low blood cell counts, and constitutional symptoms. Diagnosis involves blood tests and a bone marrow biopsy showing fibrosis. Risk stratification guides treatment, which may include drugs, splenectomy, stem cell transplant depending on disease severity and patient fitness. JAK inhibitors like ruxolitinib are first-line for symptomatic patients not eligible for transplant.

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    18 views14 pages

    MYELOFIBROSIS Final Yr 2020

    Uploaded by

    HEMA
    Myelofibrosis is a clonal hematologic malignancy characterized by the replacement of normal bone marrow with fibrous tissue. It can develop primarily (called primary myelofibrosis) or secondary to other hematologic malignancies or diseases. The main symptoms include an enlarged spleen, low blood cell counts, and constitutional symptoms. Diagnosis involves blood tests and a bone marrow biopsy showing fibrosis. Risk stratification guides treatment, which may include drugs, splenectomy, stem cell transplant depending on disease severity and patient fitness. JAK inhibitors like ruxolitinib are first-line for symptomatic patients not eligible for transplant.

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    of 14

    MYELOFIBROSIS

    DR NAILA RAZA
    MYELOFIBROSIS
    Replacement of normal hemopoietic cells by fibrous tissue.

    Primary Causes Secondary Causes


    HEMATOLOGICAL MALIGNANCIES
    Primary myelofibrosis
    (CML, ET, PV, Multiple Myeloma,
    (PMF) Lymphomas)
    METASTATIC
    (CA breast, lung or prostate)
    INFECTIVE
    (TB, fungal infections, HIV)
    METABOLIC
    (Gaucher’s disease)
    RADIATION
    OTHERS
    (Sarcoidosis, congenital
    osteopetrosis)
    Myeloproliferative Neoplasm
    • MPNs are characterized by excessive
    production of terminally differentiated blood
    cells that are fully functional.
    • Classical MPNs have been classified into 3
    entities:
    • Polycythemia vera (PV)
    • Essential thrombocythemia (ET)
    • Primary myelofibrosis (PMF) 
    PRIMARY MYELOFIBROSIS
    • A clonal hematologic malignancy with secondary (non-
    clonal) hyperproliferation of fibroblasts due to growth
    factor release(PDGF, EGF, TGF-,calreticulin) from myeloid
    cells e.g. megakaryocytes,monocytes and platelets.
    ETIOLOGY
    GENETIC MUTATIONS FREQUENCY CONSEQUENCE
    JAK2: 50-60% Increased RBC, platelet
    & granulocyte production
    CALR : 25-30% Increased platelet production 
    MPL : 3-5% Increased platelet
    production 
    CHEMICALS
    RADIATION
    PATHOGENESIS IN MYELOFIBROSIS

    6
    CLINICAL RELEVANCE OF THE
    PROGRESSIVE PATHOLOGY OF
    MYELOFIBROSIS
    •Leukoerythroblastic
    picture
    • Tear drop cells

    7
    SYMPTOMS OF MYELOFIBROSIS

    •CYTOKINE RELEASE
    •NEUTROPENIA

    MARROWEXPANSION
    OSTEOSCLEROSIS
    PERIOSTEITIS

    CYTOKINE RELEASE &


    MAST CELL
    DEGRANULATION

    CONSTITUTIONAL
    SYMPTOM

    ANEMIA
    CACHEXIA
    SPLENOMEGALY

    8
    LAB DIAGNOSIS

    • CBC
    Leucoerythroblastic blood film
    Tear drops red cells

    • BONE MARROW ASPIRATION


    Dry tap
    • BONE MARROW TREPHINE
    Fibrotic hypercellular marrow

    • JAK2 (V617F) MUTATION


    detected in approximately 50% of
    cases
    PERIPHERAL BLOOD AND BONE MARROW IN
    MYELOFIBROSIS

    tear-drop cells leukoerythroblastic picture

    Bone marrow fibrosis Reticulin stain


    WHO DIAGNOSTIC CRITERIA FOR PMF
    All major criteria plus ≥2 minor WHO criteria are required for diagnosis

    11
    RISK STRATIFICATION IN PMF

    12
    THERAPEUTIC OPTIONS

     Transplant options  Non-transplant options


    – Myeloablative • Conventional
    – Reduced-intensity – Treatment for anemia
    • Transfusion support
    • Erythropoietin
    • Corticosteriods
    • Androgen + prednisone
    Experimental drug therapy • IMiDs
    – Treatment for splenomegaly
    1. Pomalidomide • Hydroxyurea
    2. Novel JAK inhibitors • Splenectomy
    • Low-dose irradiation

    • FIRST APPROVED
    MEDICATION
    • RUXOLITINIB
    SELECTION OF UPFRONT THERAPY

    Patient Factors
    NO YES
    Advanced age
    NO YES
    Poor performance status
    Benefits NO YES Benefits
    •Curative Potential Prohibitive co-morbidities •Usually well-
    tolerated
    Disease Factors • Good QOL
    NO Severe complications YES JAK
    JAK inhibitor
    inhibitor
    HCT
    HCT of MF such as therapy/
    therapy/
    portal hypertension clinical
    clinical trial
    trial
    YES NO
    High-risk of
    Risks Risks
    • Risk of early mortality leukemic transformation
    •Unknown long-term effects
    • QOL •Duration of response ?
    • GvHD Transplant Factors •Possible resistance
    • Recurrent infections YES NO
    Well-matched donor

    Gupta V, et al. Blood 2012;120:1367-1379.

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