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    Secondary Myelofibrosis - Lagmay (3-1)

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    Secondary Myelofibrosis - Lagmay (3-1)

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    angeljeizaac
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    Original Title

    SECONDARY MYELOFIBROSIS_LAGMAY(3-1)

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    Secondary Myelofibrosis - Lagmay (3-1)

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    angeljeizaac

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    Download as pptx, pdf, or txt
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    SECONDARY

    MYELOFIBROSIS
    LAGMAY, ZHAINA MAAN S.
    BSMLS 3-1
    SECONDARY
    MYELOFIBROSI
    S
    • is a chronic disorder caused by
    other myeloproliferative
    neoplasms (MPNs), including
    essential thrombocythemia (ET)
    and polycythemia vera (PV).

    • The scarring (fi brosis) of the bone


    marrow causes reduced blood cell
    production, growing diseases, and
    potential consequences such as
    splenomegaly and anemia.
    SECONDARY
    MYELOFIBROSIS
    Conditions that can lead to secondary myelofi brosis include:
    • Polycythemia vera: A disorder in which the bone marrow makes too many
    blood cells
    • Essential Thrombocythemia: A rare chronic blood cancer
    (myeloproliferative neoplasm) characterised by the overproduction of
    platelets (thrombocytes) by megakaryocytes in the bone marrow.
    • Multiple myeloma: A rare type of blood cancer involving immune-system
    cells called plasma cells
    • Leukemia: Cancers of the white blood cells
    • Lymphoma: Cancers of lymphatic system cells
    • Radiation exposure
    • Metastatic cancer: Cancer that has spread from its origin to other tissues
    MORPHOLOGY
    Bone Marrow (BM)
    Biopsy :
    • FIBROSIS REPLACES NORMAL MARROW CELLS, LEADING TO A HYPOCELLULAR BONE MARROW IN

    LATER STAGES.

    • MEGAKARYOCYTIC HYPERPLASIA: INCREASED AND ABNORMALLY SHAPED MEGAKARYOCYTES,

    CONTRIBUTING TO FIBROSIS.

    • RETICULIN AND COLLAGEN FIBERS ARE HIGHLIGHTED WITH SPECIAL STAINS, CONFIRMING BONE

    MARROW FIBROSIS.

    Peripheral Blood Smear


    • FEATURES FIBROSIS, HYPERCELLULARITY IN EARLY STAGES, OFTEN FOLLOWING A HISTORY OF

    ESSENTIAL THROMBOCYTHEMIA (ET) OR POLYCYTHEMIA VERA (PV).

    (PBS):
    • TEARDROP-SHAPED RED CELLS (DACROCYTES), IMMATURE GRANULOCYTES, AND NUCLEATED RED

    BLOOD CELLS.

    • Leukoerythroblastosis: the presence of immature red and white blood cells.


    Pathophysiology / Abnormality
    • Arises as a progression of essential thrombocythemia (ET)
    or polycythemia vera (PV).
    • - Similar mutations as PMF (JAK2 V617F, CALR, MPL), but
    results from clonal evolution of ET or PV, leading to fibrosis.

    • Extramedullary hematopoiesis occurs as blood


    production shifts to organs like the spleen and liver.
    SIGNS AND
    SYMPTOMS
    • General symptoms:
    ⚬ Fatigue, weakness, night sweats, and weight loss due to systemic
    inflammation.
    • Splenomegaly:
    ⚬ Enlarged spleen causing abdominal discomfort, early satiety, and
    left upper quadrant pain.
    • Anemia:
    ⚬ Pallor, shortness of breath, and tiredness, often due to decreased
    red blood cell production.
    • Bleeding or bruising:
    ⚬ Abnormal platelet function, leading to easy bruising or bleeding.
    • Bone pain and discomfort.
    •
    LABORATORY DIAGNOSIS
    • Complete Blood Count (CBC):
    ⚬ Anemia is common, often with variable white blood cell
    (WBC) and platelet counts (thrombocytosis early on,
    thrombocytopenia in advanced stages).
    • Peripheral Blood Smear:
    ⚬ Shows teardrop-shaped red cells, immature white cells, and
    nucleated red cells.
    • Bone Marrow Biopsy:
    ⚬ Reticulin stain to confirm fibrosis, with increased and
    atypical megakaryocytes.
    ⚬ The degree of fibrosis is graded, often using a scale of 0-3.
    • Molecular Testing:
    Testing for JAK2, CALR, and MPL mutations helps confirm the
    diagnosis and guides treatment.
    TREATMENTS
    • Symptom management:
    ⚬ Ruxolitinib (JAK1/2 inhibitor) is a first-line treatment that helps
    reduce spleen size and improve symptoms like fatigue and night
    sweats.
    ⚬ Fedratinib, another JAK inhibitor, may be used if Ruxolitinib is not
    effective.
    • Supportive treatments:
    ⚬ Blood transfusions for severe anemia.
    ⚬ Erythropoiesis-stimulating agents (ESAs) to help increase red
    blood cell production in anemic patients.
    ⚬ Hydroxyurea: Used to control elevated blood cell counts in earlier
    phases of the disease.
    • Stem cell transplantation:
    ⚬ The only potentially curative option but limited to younger patients
    with a good performance status due to the high risks associated with
    transplantation.
    • Clinical trials:
    ⚬ New treatments targeting the fibrotic process, such as telomerase
    inhibitors (Imetelstat) and antifibrotic agents, are being
    PROGNOSIS AND OTHER
    INFORMATION
    Dependent on the stage at which the progression from ET or PV is

    identified.

    - Generally worse prognosis than original MPN, with median survival

    of 2-5 years in high-risk patients.

    - Risk of AML transformation higher in SMF compared to PV or ET

    alone.
    REFERENCES
    :
    Moawad, H. (2022, August 17). Facts about
    https://www.verywellhealth.com/facts-about-myelofibrosis-5667212
    myelofibrosis. Verywell Health.

    Massive Bio. (n.d.). Secondary myelofibrosis. Retrieved from


    https://massivebio.com/secondary-myelofibrosis/#:~:text=Secondary%20myelofibrosis%20is%20a%20grou
    p,cells%20or%20mesenchymal%20stem%20cells

    Tefferi, A. & Pardanani, A. (2019). Myeloproliferative Neoplasms: 2019 Update on Diagnosis, Risk-
    Stratification, and Management. American Journal of Hematology, 94(1), 133-149.

    Mesa, R. A., et al. (2017). International Working Group for Myelofibrosis Research and Treatment (IWG-
    MRT) Consensus Report. Blood, 129(5), 680-692.

    Reilly, J. T., et al. (2012). A European LeukemiaNet Study: Primary vs. Secondary Myelofibrosis, Survival
    and Prognostic Factors. Blood, 119(11), 2563-2569.

    Vannucchi, A. M., & Harrison, C. (2017). Emerging Treatments for Myelofibrosis: JAK Inhibitors and Beyond.
    Blood, 129(6), 693-702.

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