LEUKEMIA...ALL U NEED TO KNOW

Discussion in 'AIIMS Nov 2013' started by samuel, Aug 21, 2014.

  1. samuel

    samuel New Member

    Hemophagocytic lymphohistiocytosis (HLH)
    is a reactive condition marked by cytopenias and signs and symptoms of systemic inflammation related to macrophage activation.

    it is also sometimes referred to as macrophage activation syndrome.

    familial and may appear early in life, even in infants, while other forms are
    sporadic and may affect people of any age.

    The common feature of all forms of HLH issystemic activation of macrophages and CD8+ cytotoxic T cells.


    the “stew” of mediators released from macrophages and lymphocytes suppress hematopoiesis and produce symptoms of systemic inflammation. These effects lead to cytopenias and a shock-like picture, sometimes referred to as “cytokine storm” or the systemic inflammatory response syndrome

    Clinical Features.
    Most patients present with an acute febrile illness associated with splenomegaly and hepatomegaly. Hemophagocytosis is usually seen on bone marrow examination, but is neither sufficient nor required to make the diagnosis.

    Laboratory studies
    reveal anemia, thrombocytopenia, and very high levels of plasma
    ferritin and soluble IL-2 receptor.Coagulation
    studies may show evidence of disseminated intravascular
    coagulation.

    Treatment involves the use of immunosuppressive
    drugs and “mild” chemotherapy. Patients with germline
    mutations that cause HLH or who have persistent/resistant
    disease are candidates for hematopoietic stem cell
    transplantation.
    familial forms of the disease,
    who typically survive for less than 2 months.
  2. samuel

    samuel New Member

    POLYCYTHEMIA VERA

    `` JAK2 V617F mutation.
    `` Increased red blood cell mass.
    `` Splenomegaly.
    `` Normal arterial oxygen saturation.
    `` Usually elevated white blood count and platelet count.
  3. samuel

    samuel New Member

    Causes of polycythemia.

    Spurious polycythemia
    Secondary polycythemia
    Hypoxia: cardiac disease, pulmonary disease, high altitude
    Carboxyhemoglobin: smoking
    Kidney lesions
    Erythropoietin-secreting tumors (rare)
    Abnormal hemoglobins (rare)
    Polycythemia vera
  4. samuel

    samuel New Member

    ESSENTIAL THROMBOCYTOSIS

    `` Elevated platelet count in absence of other causes.
    `` Normal red blood cell mass.
    `` Absence of bcr/abl gene (Philadelphia chromosome).
  5. samuel

    samuel New Member

    Primary MYELOFIBROSIS

    `` Striking splenomegaly.
    `` Teardrop poikilocytosis on peripheral smear.
    `` Leukoerythroblastic blood picture; giant abnormal
    platelets.
    `` Hypercellular bone marrow with reticulin or
    collagen fibrosis.
  6. samuel

    samuel New Member

    CHRONIC MYELOID LEUKEMIA

    `` Elevated white blood count.
    `` Markedly left-shifted myeloid series but with a low percentage of promyelocytes and blasts.
    `` Presence of bcr/abl gene (Philadelphia chromosome).
  7. samuel

    samuel New Member

    MYELODYSPLASTIC SYNDROMES

    `` Cytopenias with a hypercellular bone marrow.
    `` Morphologic abnormalities in two or more hematopoietic cell lines.
  8. samuel

    samuel New Member

    ACUTE LEUKEMIA

    `` Short duration of symptoms, including fatigue, fever, and bleeding.
    `` Cytopenias or pancytopenia.
    `` More than 20% blasts in the bone marrow.
    `` Blasts in peripheral blood in 90% of patients.
    `` Classify as acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL).
  9. samuel

    samuel New Member

    CHRONIC LYMPHOCYTIC LEUKEMIA

    `` B-cell lymphocytosis > 5000/mcL.
    `` Coexpression of CD19, CD5 on lymphocytes.
  10. samuel

    samuel New Member

    HAIRY CELL LEUKEMIA

    `` Pancytopenia.
    `` Splenomegaly, often massive.
    `` Hairy cells present on blood smear and especially in bone marrow biopsy.
  11. samuel

    samuel New Member

    Precursor B-cell lymphoblastic lymphoma
    Mature B-cell lymphomas
    -Diffuse large B cell lymphoma
    -Mediastinal large B cell lymphoma
    -Follicular lymphoma
    -Small lymphocytic lymphoma
    -Lymphoplasmacytic lymphoma (Waldenström macroglobulinemia)
    -Mantle cell lymphoma
    -Burkitt lymphoma
    -Marginal zone lymphoma
    --MALT type
    --Nodal type
    --Splenic type
    Precursor T-cell lymphoblastic lymphoma
    Mature T (and NK cell) lymphomas
    Anaplastic T-cell lymphoma
    -Angioimmunoblastic
    -Peripheral T-cell lymphoma, NOS
    -Cutaneous T-cell lymphoma (mycosis fungoides)
    -Extranodal T/NK-cell lymphoma
    -Adult T-cell leukemia/lymphoma

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