Transcript Splenectomy in Hematologic Disorders

Splenectomy in
Hematologic Disorders
Scott Nguyen
Team 4
6/11/04
Indications
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Idiopathic Thrombocytopenic Purpura (ITP)
Hereditary Spherocytosis
Chronic Autoimmune Hemolytic Anemia
Non Hodgkins Lymphoma
Hairy Cell Leukemia
Chronic Lymphocytic Lymphoma / Chronic
Myelogenous Lymphoma
Chronic ITP
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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
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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
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ITP - Management
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Assx disease w/ Plts > 50K – no tx needed
If < 50K, bleeding – tx indicated
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Glucocorticoids – Prednisone 1mg/kg
2/3 respond initially
 25% completely respond
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Acute severe bleeding
Platelet transfusions
 IVIG
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Refractory ITP
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Most respond to steroids, but >75% pts recurr after
steroids tapered
Splenectomy – removes source of antiplatelet Ig,
removes source of phagocytic cells
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Indications –
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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
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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
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Missed accessory spleen in 10%
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Can localize w/ Radionuclide imaging
Long term steroid therapy
Azathioprine or cyclophosphamide
Hereditary Spherocytosis
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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
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Clinical presentation – anemia, jaundice,
cholelithiasis, splenomegaly
Diagnosis –
Family history of anemia
 spherocytes on peripheral smear
 Increased reticulocyte count
 Increased osmotic fragility
 Negative Coombs test
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Treatment
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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
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Conditions in which autoantibodies against pt’s
own red cells are formed (IgG)
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Abnormal destruction
“marked” RBCs are prematurely destroyed by
phagocytic cells
 Complement activated on cell membranes –
resulting in lysis
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Causes
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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
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More in adults, F > M
Moderate to severe anemia, high reticulocytes
Spherocytosis on blood smears
Splenomegaly
Direct Coomb test positive
Direct Coombs test
Treatment
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Make sure not drug related
Steroids –
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75% respond, 50% relapse
Splenectomy for those who fail steroid therapy
Refractory cases – azathioprine and
cyclophosphamide, other immunosuppressive
drugs
Hodgkins Lymphoma
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Splenectomy routinely performed during staging
laparotomy
Confirms disease below the diaphragm (upstages
II -> III)
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Radiation tx -> Chemotx
Historically performed often
Advances in imaging – CT, lymphangiography,
PET – much improved nonoperative staging
Hairy Cell Leukemia
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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
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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
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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
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Splenectomy
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CML and CLL
Pyruvate Kinase deficiency
Hemoglobinopathies – sickle cell, thalassemia