Splenectomy in Hematologic Disorders

Posted by e-Medical PPT Tuesday, May 1, 2012
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
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

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

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..

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