Splenectomy in Splenectomy in Hematologic Hematologic

DisordersDisordersScott NguyenScott Nguyen

Team 4Team 4

6/11/046/11/04

IndicationsIndications

Idiopathic Thrombocytopenic Purpura Idiopathic Thrombocytopenic Purpura (ITP)(ITP)

Hereditary SpherocytosisHereditary Spherocytosis Chronic Autoimmune Hemolytic Chronic Autoimmune Hemolytic

AnemiaAnemia Non Hodgkins LymphomaNon Hodgkins Lymphoma Hairy Cell LeukemiaHairy Cell Leukemia Chronic Lymphocytic Lymphoma / Chronic Lymphocytic Lymphoma /

Chronic Myelogenous LymphomaChronic Myelogenous Lymphoma

Chronic ITPChronic ITP

Autoimmune disorder of AdultsAutoimmune disorder of Adults Autoantibodies to platelet Autoantibodies to platelet

glycoproteins glycoproteins Antibodies act as opsonins and Antibodies act as opsonins and

accelerate platelet clearance by accelerate platelet clearance by phagocytic cellsphagocytic cells

Also can bind to critical regions of the Also can bind to critical regions of the glycoproteins and impair functionglycoproteins and impair function

F > M 3:1, ages 20-40 F > M 3:1, ages 20-40

Chronic ITPChronic ITP

Purpura, epistaxis, gingival bleedingPurpura, epistaxis, gingival bleeding Rarely GI, GU, intracranial hemorrhageRarely GI, GU, intracranial hemorrhage Diagnosis – low platelet count, normal Diagnosis – low platelet count, normal

bone marrow, exclusion of other causes bone marrow, exclusion of other causes of thrombocytopeniaof thrombocytopenia

Drugs Drugs Viral infections Viral infections Autoimmune diseasesAutoimmune diseases Lymphoproliferative diseasesLymphoproliferative diseases

ITP - ManagementITP - Management

Assx disease w/ Plts > 50K – no tx Assx disease w/ Plts > 50K – no tx neededneeded

If < 50K, bleeding – tx indicatedIf < 50K, bleeding – tx indicated Glucocorticoids – Prednisone 1mg/kgGlucocorticoids – Prednisone 1mg/kg

2/3 respond initially2/3 respond initially 25% completely respond25% completely respond

Acute severe bleedingAcute severe bleeding Platelet transfusionsPlatelet transfusions IVIG IVIG

Refractory ITPRefractory ITP

Most respond to steroids, but >75% pts Most respond to steroids, but >75% pts recurr after steroids taperedrecurr after steroids tapered

Splenectomy – removes source of antiplatelet Splenectomy – removes source of antiplatelet Ig, removes source of phagocytic cellsIg, removes source of phagocytic cells Indications – Indications –

Plts < 10K after 6 wks med txPlts < 10K after 6 wks med tx Plts < 30K, had insuffic response to med tx after 3mosPlts < 30K, had insuffic response to med tx after 3mos Emergent splenectomy in cases of intracranial bleedingEmergent splenectomy in cases of intracranial bleeding

Platelet transfusions should only be given Platelet transfusions should only be given after splenic artery ligated to prevent after splenic artery ligated to prevent destructiondestruction

Splenectomy for ITPSplenectomy for ITP

65-80% successful65-80% successful Usually platelet counts respond by Usually platelet counts respond by

10 days10 days Age < 60, good inititial response to Age < 60, good inititial response to

steroids are favorable factorssteroids are favorable factors Laparoscopic splenectomy popular Laparoscopic splenectomy popular

as spleen is usually small to normal as spleen is usually small to normal sized sized

Unsuccessful Unsuccessful SplenectomySplenectomy

Missed accessory spleen in 10%Missed accessory spleen in 10% Can localize w/ Radionuclide imagingCan localize w/ Radionuclide imaging

Long term steroid therapyLong term steroid therapy Azathioprine or cyclophosphamideAzathioprine or cyclophosphamide

Hereditary SpherocytosisHereditary Spherocytosis

Autosomal dominant deficiency of Autosomal dominant deficiency of spectrin, red cell cytoskeletal spectrin, red cell cytoskeletal protein - maintains osmotic stabilityprotein - maintains osmotic stability

Membrane abnormality results in Membrane abnormality results in red cells which are small, spherical, red cells which are small, spherical, and rigidand rigid

Spherocytes more susceptible to Spherocytes more susceptible to becoming trapped in spleen and becoming trapped in spleen and destroyeddestroyed

Hereditary SpherocytosisHereditary Spherocytosis

Hereditary SpherocytosisHereditary Spherocytosis

Clinical presentation – anemia, Clinical presentation – anemia, jaundice, cholelithiasis, splenomegalyjaundice, cholelithiasis, splenomegaly

Diagnosis – Diagnosis – Family history of anemiaFamily history of anemia spherocytes on peripheral smearspherocytes on peripheral smear Increased reticulocyte countIncreased reticulocyte count Increased osmotic fragilityIncreased osmotic fragility Negative Coombs testNegative Coombs test

TreatmentTreatment

Splenectomy decreases rate of Splenectomy decreases rate of hemolysishemolysis

If diagnosed in childhood, If diagnosed in childhood, splenectomy should wait until after splenectomy should wait until after 4yo to preserve immunologic 4yo to preserve immunologic function of spleenfunction of spleen

Cholecystectomy can be performed Cholecystectomy can be performed at same operation if documented at same operation if documented gallstonesgallstones

Autoimmune Hemolytic Autoimmune Hemolytic AnemiaAnemia

Conditions in which autoantibodies Conditions in which autoantibodies against pt’s own red cells are formed against pt’s own red cells are formed (IgG)(IgG)

Abnormal destructionAbnormal destruction ““marked” RBCs are prematurely marked” RBCs are prematurely

destroyed by phagocytic cellsdestroyed by phagocytic cells Complement activated on cell Complement activated on cell

membranes – resulting in lysismembranes – resulting in lysis

CausesCauses

IdiopathicIdiopathic Lymphoproliferative diseases – CLL, Lymphoproliferative diseases – CLL,

NHL, Hodgkins diseaseNHL, Hodgkins disease Systemic Lupus Erythematosus or Systemic Lupus Erythematosus or

other Collagen Vascular diseasesother Collagen Vascular diseases Postviral infectionsPostviral infections Drug induced (methyldopa, pcn, Drug induced (methyldopa, pcn,

quinidine)quinidine)

Autoimmune hemolytic Autoimmune hemolytic anemiaanemia

More in adults, F > MMore in adults, F > M Moderate to severe anemia, high Moderate to severe anemia, high

reticulocytesreticulocytes Spherocytosis on blood smearsSpherocytosis on blood smears SplenomegalySplenomegaly Direct Coomb test positiveDirect Coomb test positive

Direct Coombs testDirect Coombs test

TreatmentTreatment

Make sure not drug related Make sure not drug related Steroids – Steroids –

75% respond, 50% relapse75% respond, 50% relapse Splenectomy for those who fail Splenectomy for those who fail

steroid therapy steroid therapy Refractory cases – azathioprine and Refractory cases – azathioprine and

cyclophosphamide, other cyclophosphamide, other immunosuppressive drugsimmunosuppressive drugs

Hodgkins LymphomaHodgkins Lymphoma

Splenectomy routinely performed Splenectomy routinely performed during staging laparotomyduring staging laparotomy

Confirms disease below the Confirms disease below the diaphragm (upstages II -> III)diaphragm (upstages II -> III) Radiation tx -> ChemotxRadiation tx -> Chemotx

Historically performed often Historically performed often Advances in imaging – CT, Advances in imaging – CT,

lymphangiography, PET – much lymphangiography, PET – much improved nonoperative stagingimproved nonoperative staging

Hairy Cell LeukemiaHairy Cell Leukemia

2% adult leukemias2% adult leukemias Hairy Cells – neoplastic B lymphocytes Hairy Cells – neoplastic B lymphocytes

w/ cell membrane rufflingw/ cell membrane ruffling Found in peripheral blood and bone Found in peripheral blood and bone

marrowmarrow Usually in elderly men Usually in elderly men Symptoms d/t pancytopenia from Symptoms d/t pancytopenia from

hypersplenism and infiltration of bone hypersplenism and infiltration of bone marrowmarrow

Hairy Cell LeukemiaHairy Cell Leukemia

Hairy Cell LeukemiaHairy Cell Leukemia

Massive splenomegaly causing Massive splenomegaly causing hypersplenism – rapidly and hypersplenism – rapidly and prematurely destroy all blood cells prematurely destroy all blood cells

Symptomatic anemiaSymptomatic anemia Infectious complications from Infectious complications from

neutropenianeutropenia Bleeding complications from Bleeding complications from

thrombocytopeniathrombocytopenia Increased risk of second malignancyIncreased risk of second malignancy

TreatmentTreatment

Alpha interferon, purine analogsAlpha interferon, purine analogs Splenectomy in refractory casesSplenectomy in refractory cases

40% pts get normalization of blood 40% pts get normalization of blood counts aftercounts after

Usually response lasts > 10 yrs Usually response lasts > 10 yrs 50% pts require no further tx50% pts require no further tx

CML and CLL CML and CLL Pyruvate Kinase deficiencyPyruvate Kinase deficiency Hemoglobinopathies – sickle cell, Hemoglobinopathies – sickle cell,

thalassemiathalassemia

SplenectomySplenectomy