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Splenectomy in Hematologic Disorders

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					  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
                 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
                    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
                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
                       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
            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
        Unsuccessful Splenectomy
   Missed accessory spleen in 10%
       Can localize w/ Radionuclide imaging
   Long term steroid therapy
   Azathioprine or cyclophosphamide
        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
Hereditary Spherocytosis
        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
                  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
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
                    Causes
   Idiopathic
   Lymphoproliferative diseases – CLL, NHL,
    Hodgkins disease
   Systemic Lupus Erythematosus or other
    Collagen Vascular diseases
   Postviral infections
   Drug induced (methyldopa, pcn, quinidine)
    Autoimmune hemolytic anemia
   More in adults, F > M
   Moderate to severe anemia, high reticulocytes
   Spherocytosis on blood smears
   Splenomegaly
   Direct Coomb test positive
Direct Coombs test
                     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
            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
           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
Hairy Cell Leukemia
           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
                    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
                 Splenectomy


   CML and CLL
   Pyruvate Kinase deficiency
   Hemoglobinopathies – sickle cell, thalassemia

				
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