Splenic Neoplasms

• • 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

Epidemiology

Chronic Lymphocytic Leukemia (CLL)

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

Hairy Cell Leukemia (HCL)

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

  • Patients are typically middle aged men

Idiopathic Myelofibrosis

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

Mast Cell Disease (MCD)

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

Symptoms and Signs

CLL

• • Fatigue

  • Splenomegaly

  • Lymphadenopathy

  • Bleeding

HCL

• • Splenomegaly

  • Infections

  • Bleeding

  • Fatigue

Idiopathic Myelofibrosis

• • Weakness and fatigue

  • Dyspnea

  • Splenomegaly

  • Abdominal fullness and pain

  • Bleeding

  • Infection

MCD

• • Splenomegaly

  • Bleeding

Laboratory Findings

CLL

• • Lymphocytosis

  • Thrombocytopenia and anemia

HCL

• • Pancytopenia

  • Hairy cells on bone marrow biopsy and peripheral smear

Idiopathic Myelofibrosis

• • Anemia

  • Leukocytosis or leukopenia

  • Thrombocytosis or thrombocytopenia

  • Leukoerythroblastic peripheral smear with teardrop poikilocytosis

  • Giant abnormal platelets

MCD

• • Thrombocytopenia

CLL, HCL, and MCD

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

Idiopathic Myelofibrosis

• • 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

Rule Out

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

• • CBC

  • Peripheral blood smear

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

When to Admit

• • Severe anemia, leukemia, thrombocytopenia

  • Bleeding

When to Refer

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

Surgery

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