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Splenectomy in Hematologic Disorders

Splenectomy in Hematologic Disorders. Scott Nguyen Team 4 6/11/04. Indications. Idiopathic Thrombocytopenic Purpura (ITP) Hereditary Spherocytosis Chronic Autoimmune Hemolytic Anemia Non Hodgkins Lymphoma Hairy Cell Leukemia Chronic Lymphocytic Lymphoma / Chronic Myelogenous Lymphoma.

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Splenectomy in Hematologic Disorders

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  1. Splenectomy in Hematologic Disorders Scott Nguyen Team 4 6/11/04

  2. Indications • Idiopathic Thrombocytopenic Purpura (ITP) • Hereditary Spherocytosis • Chronic Autoimmune Hemolytic Anemia • Non Hodgkins Lymphoma • Hairy Cell Leukemia • Chronic Lymphocytic Lymphoma / Chronic Myelogenous Lymphoma

  3. Chronic ITP • Autoimmune disorder of Adults • Autoantibodies to platelet glycoproteins • Antibodies act as opsonins and accelerate platelet clearance by phagocytic cells • Also can bind to critical regions of the glycoproteins and impair function • F > M 3:1, ages 20-40

  4. Chronic ITP • Purpura, epistaxis, gingival bleeding • Rarely GI, GU, intracranial hemorrhage • Diagnosis – low platelet count, normal bone marrow, exclusion of other causes of thrombocytopenia • Drugs • Viral infections • Autoimmune diseases • Lymphoproliferative diseases

  5. ITP - Management • Assx disease w/ Plts > 50K – no tx needed • If < 50K, bleeding – tx indicated • Glucocorticoids – Prednisone 1mg/kg • 2/3 respond initially • 25% completely respond • Acute severe bleeding • Platelet transfusions • IVIG

  6. Refractory ITP • Most respond to steroids, but >75% pts recurr after steroids tapered • Splenectomy – removes source of antiplatelet Ig, removes source of phagocytic cells • Indications – • Plts < 10K after 6 wks med tx • Plts < 30K, had insuffic response to med tx after 3mos • Emergent splenectomy in cases of intracranial bleeding • Platelet transfusions should only be given after splenic artery ligated to prevent destruction

  7. Splenectomy for ITP • 65-80% successful • Usually platelet counts respond by 10 days • Age < 60, good inititial response to steroids are favorable factors • Laparoscopic splenectomy popular as spleen is usually small to normal sized

  8. Unsuccessful Splenectomy • Missed accessory spleen in 10% • Can localize w/ Radionuclide imaging • Long term steroid therapy • Azathioprine or cyclophosphamide

  9. Hereditary Spherocytosis • Autosomal dominant deficiency of spectrin, red cell cytoskeletal protein - maintains osmotic stability • Membrane abnormality results in red cells which are small, spherical, and rigid • Spherocytes more susceptible to becoming trapped in spleen and destroyed

  10. Hereditary Spherocytosis

  11. Hereditary Spherocytosis • Clinical presentation – anemia, jaundice, cholelithiasis, splenomegaly • Diagnosis – • Family history of anemia • spherocytes on peripheral smear • Increased reticulocyte count • Increased osmotic fragility • Negative Coombs test

  12. Treatment • Splenectomy decreases rate of hemolysis • If diagnosed in childhood, splenectomy should wait until after 4yo to preserve immunologic function of spleen • Cholecystectomy can be performed at same operation if documented gallstones

  13. Autoimmune Hemolytic Anemia • Conditions in which autoantibodies against pt’s own red cells are formed (IgG) • Abnormal destruction • “marked” RBCs are prematurely destroyed by phagocytic cells • Complement activated on cell membranes – resulting in lysis

  14. Causes • Idiopathic • Lymphoproliferative diseases – CLL, NHL, Hodgkins disease • Systemic Lupus Erythematosus or other Collagen Vascular diseases • Postviral infections • Drug induced (methyldopa, pcn, quinidine)

  15. Autoimmune hemolytic anemia • More in adults, F > M • Moderate to severe anemia, high reticulocytes • Spherocytosis on blood smears • Splenomegaly • Direct Coomb test positive

  16. Direct Coombs test

  17. Treatment • Make sure not drug related • Steroids – • 75% respond, 50% relapse • Splenectomy for those who fail steroid therapy • Refractory cases – azathioprine and cyclophosphamide, other immunosuppressive drugs

  18. Hodgkins Lymphoma • Splenectomy routinely performed during staging laparotomy • Confirms disease below the diaphragm (upstages II -> III) • Radiation tx -> Chemotx • Historically performed often • Advances in imaging – CT, lymphangiography, PET – much improved nonoperative staging

  19. Hairy Cell Leukemia • 2% adult leukemias • Hairy Cells – neoplastic B lymphocytes w/ cell membrane ruffling • Found in peripheral blood and bone marrow • Usually in elderly men • Symptoms d/t pancytopenia from hypersplenism and infiltration of bone marrow

  20. Hairy Cell Leukemia

  21. Hairy Cell Leukemia • Massive splenomegaly causing hypersplenism – rapidly and prematurely destroy all blood cells • Symptomatic anemia • Infectious complications from neutropenia • Bleeding complications from thrombocytopenia • Increased risk of second malignancy

  22. Treatment • Alpha interferon, purine analogs • Splenectomy in refractory cases • 40% pts get normalization of blood counts after • Usually response lasts > 10 yrs • 50% pts require no further tx

  23. Splenectomy • CML and CLL • Pyruvate Kinase deficiency • Hemoglobinopathies – sickle cell, thalassemia

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