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Gaucher's Disease

This autosomal recessive disorder, which is caused by a deficiency of glucocerebrosidase, occurs primarily in Ashkenazi Jews. It results in massive splenomegaly from an accumulation of insoluble glucosylceramide in the spleen. Diagnosis is made by determination of leukocyte beta-glucosidase activity. As the process evolves, patients may develop abnormal liver function, hepatic fibrosis, anemia, thrombocytopenia,

and bone involvement. Splenectomy is helpful in managing symptomatic splenomegaly and cytopenias. Partial splenectomy has been performed in an attempt to lessen the risk of postsplenectomy infection and bone crises. However, after partial splenectomy, symptomatic splenic enlargement and worsening hypersplenism recur after 3 to 8 years and necessitate later total splenectomy- Partial splenectomy may be considered as a temporizing procedure in young children.

Wiskott-Aldrich Syndrome

Wiskott-Aldrich syndrome is a hereditary immunodeficiency that presents with thrombocytopenia and recurrent infections. Splenectomy brings about a sustained rise in platelet count without accentuation of the continued risk of repeated infections.1 • Congestive Splenomegaly

Portal hypertension from any cause may be accompanied by thrombocytopenia induced by slower blood flow through congested splenic sinuses. Splenectomy alone for the management of thrombocytopenia associated with hepatic cirrhosis and bleeding esophageal varices is inappropriate because platelet counts usually rise to safe levels after one of the portal-systemic shunting procedures. Removal of the spleen is reserved for patients with left-sided portal hypertension and hypersplenism resulting from splenic vein thrombosis and for the rare patient with persistent severe thrombocytopenia after a portal-systemic shunt. Several patients with idiopathic splenomegaly and hypersplenism have been diagnosed as having a mild form of hepatic cirrhosis only at the time of splenectomy and concomitant liver biopsy.

Thrombotic Thrombocytopenic Purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare condition that has engendered a large body of literature related to pathogenesis and treatment. TTP is characterized by fever, hemolytic anemia, thrombocytopenia, fluctuating neurologic changes, schistocytes (fragmented red blood cells) in peripheral blood, microvascular thromboses (arterioles, capillaries, venules) in many organs, and, in some patients, renal insufficiency. TTP, which usually occurs in adults, is related in some aspects to the microangiopathic hemolytic anemia of the hemolytic-uremic syndrome, which occurs primarily in children.

The primary pathologic change in TTP appears to be damage to the vascular endothelium of small blood vessels. Exposure of subendothelial collagen then brings about platelet adhesion and aggregation, with initiation of thrombosis. The reason for confinement of this process primarily to small blood vessels has not been explained. The pathogenesis of endothelial damage has been attributed to bacterial or viral infections, endotoxins (with possible secondary formation of immune complexes), cancer, or primary autoimmune conditions, but no definite cause has been proven. Some possible coagulative abnormalities have been reported (rapid loss of prostacyclin activity, a new platelet-aggregating factor, increased large multimers of factor VIII—von Willebrand factor), but whether these alterations in coagulation are consistent and whether they represent cause or effect will require further studies.

Most patients who present with TTP are otherwise healthy young or middle-aged adults without a demonstrable underlying disease. Studies of peripheral blood usually demonstrate the presence of severe thrombocytopenia, schistocytes, and a variable degree of hemolytic anemia. Elevation of serum lactic dehydrogenase levels is thought to correlate with the severity of the thrombotic process.1 ' In doubtful cases, gingival biopsy has been used to demonstrate fibrin-platelet thrombi in small vessels.

Because this condition is relatively rare, no randomized controlled trials of various treatment modalities have been conducted. However, nonoperative measures have markedly lowered the original high mortality from this disease. Plasmapheresis has had superior therapeutic success under the premise that such a procedure removes immune complexes or some other toxic factor.1 • However, some patients respond to infusion of fresh frozen plasma alone, provided the volume of plasma infused does not induce symptomatic hypervolemia. Hemodialysis may be required if renal insufficiency is severe. Corticosteroids, aspirin, and dipyridamole have also been administered also to most patients under the premises that this is an immunologically mediated condition and that antiplatelet drugs may inhibit platelet aggregation. Several

anecdotal reports suggest that platelet transfusions may worsen the manifestations of this disease. Splenectomy is reserved usually for patients who fail to obtain an adequate response to the foregoing treatments. In my experience, splenectomy has resulted in a dramatic improvement in some patients. An occasional patient responds to initial treatment and then has a relapse months to years later. Others develop a low-grade chronic process that also improves after splenectomy. SPLENECTOMY FOR THE "WANDERING SPLEEN" AND SPLENIC TORSION

A wandering spleen results from increased splenic mobility on an elongated splenic pedicle. This condition probably results from congenital weakness or atresia of the lienorenal ligament as a result of failure of fusion of the dorsal mesogastrium, from which the spleen develops embryologically. Multiparity may serve to aggravate this ligamentous laxity. This condition may present as an asymptomatic abdominal mass or, more commonly, as an abdominal mass with associated intermittent abdominal pain. A more serious situation is presentation as an acute abdomen resulting from persistent splenic torsion and infarction. Although preoperative diagnosis has been uncommon, computed tomography (CT), spleen scan, or occasionally ultrasound studies may be diagnostic. In an acute situation, angiography can define whether occlusion of the splenic artery has occurred.

Both operative and non-operative management of an asymptomatic patient have been advocated. Patients with chronic symptoms have been treated by both splenopexy and splenectomy, whereas only the latter procedure is appropriate for acute splenic torsion and infarction.1 ' Many patients with chronic symptoms develop congestive splenomegaly, and a few have secondary hypersplenism, for which only spleen removal is suitable. In the nonemergency situation, splenopexy deserves more serious consideration in the young child, who is more susceptible to postsplenectomy sepsis.

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