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  • • Splenic involvement (splenomegaly and hypersplenism) due to infiltration of lymphocytes or mast cells or initiation of extramedullary hematopeosis

    • Splenomegaly and hypersplenism, resulting in anemia, thrombocytopenia, and/or neutropenia

    • Splenectomy may be indicated for hypersplenism, for diagnosis, or for primary surgical removal of the predominant or only site of disease

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Epidemiology

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Chronic Lymphocytic Leukemia (CLL)

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  • • Neoplasm of B cells characterized by bone marrow and splenic infiltration of lymphocytes that are mature morphologically but functionally incompetent

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Hairy Cell Leukemia (HCL)

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  • • A low-grade lymphoproliferative disorder with characteristic "hairy cells" that infiltrate the bone marrow and spleen

    • Patients are typically middle aged men

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Idiopathic Myelofibrosis

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  • • Results in extensive bone marrow fibrosis and extramedullary hematopoiesis in the spleen and liver

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Mast Cell Disease (MCD)

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  • • Rare condition characterized by mast cell infiltration of a number of tissues, including the spleen

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Symptoms and Signs

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CLL

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  • • Fatigue

    • Splenomegaly

    • Lymphadenopathy

    • Bleeding

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HCL

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  • • Splenomegaly

    • Infections

    • Bleeding

    • Fatigue

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Idiopathic Myelofibrosis

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  • • Weakness and fatigue

    • Dyspnea

    • Splenomegaly

    • Abdominal fullness and pain

    • Bleeding

    • Infection

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MCD

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  • • Splenomegaly

    • Bleeding

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Laboratory Findings

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CLL

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  • • Lymphocytosis

    • Thrombocytopenia and anemia

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HCL

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  • • Pancytopenia

    • Hairy cells on bone marrow biopsy and peripheral smear

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Idiopathic Myelofibrosis

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  • • Anemia

    • Leukocytosis or leukopenia

    • Thrombocytosis or thrombocytopenia

    • Leukoerythroblastic peripheral smear with teardrop poikilocytosis

    • Giant abnormal platelets

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MCD

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  • • Thrombocytopenia

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CLL, HCL, and MCD

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  • • Cytopenias due to a combination of bone marrow and splenic infiltration with secondary hypersplenism

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Idiopathic Myelofibrosis

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  • • Pain over the spleen from splenic infarcts is common

    • Portal hypertension develops in some cases as a result of fibrosis of the liver, greatly increased splenic blood flow, or both

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Rule Out

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  • • Other causes of hypersplenism and cytopenias (Waldenstrom macroglobulinemia, non-Hodgkin lymphoma, Hodgkin disease, chronic myelogenous leukemia)

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  • • CBC

    • Peripheral blood smear

    • Bone marrow biopsy and aspiration (may be a dry tap in idiopathic myelofibrosis due to bone marrow fibrosis)

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When to Admit

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  • • Severe anemia, leukemia, thrombocytopenia

    • Bleeding

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When to Refer

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  • • All disorders should be managed in conjunction with an hematologist

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Surgery

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  • • Splenectomy

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Indications

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  • • Splenomegaly with abdominal pain

    • Hypersplenism with cytopenias

    • Adjunct to ...

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