Splenectomy, thrombocytopenia

Management may on occasions have to be splenectomy but in these situations there are generally other factors. For example, in chronic lymphocytic leukaemia contributions to the thrombocytopenia that occurs from reduced production of the one hand and rapid clearance due to immune mechanisms on the other. 

In patients with chronic idiopathic thrombocytopenia (UP) who failed to respond adequately to previous treatment with steroids, immunoglobulins, or splenectomy, romiplostim significantly increased platelet count in most patients. In a 6-week placebo-controlled study in which patients were treated weekly with 1 or 3 mcg/kg, 12 of 16 patients reached the targeted platelet range of 50,000-450,000 platelets/mL. Romiplostim does not appear to decrease the rate of platelet destruction in ITP as platelet counts returned to pretreatment levels after the drug s discontinuation. An open label trial found that many patients maintained a platelet count of 100,000 platelets/mL or higher over a 48-week period and that over half of the patients were able to discontinue other therapies. Romiplostim is initiated as a weekly subcutaneous dose of 1 mcg/kg and then continued at the lowest dose required to maintain a platelet count of at least 50,000 platelets/mL. 

Surgical removal of tissues may be necessary in some cases to alleviate symptoms, such as oropharyngeal obstruction (tonsillectomy) and progressive anemia and thrombocytopenia (splenectomy). 

Treatment of sphingolipidoses is primarily symptomatic and supportive. For example, in patients with anemia due to Gaucher s disease, thrombocytopenia associated with hypersplenism is relieved by splenectomy. Infusion of appropriate purified human placental tissue enzymes in patients with Gaucher s disease and Fabry s disease reduced the accumulated glycolipids in the circulation and liver. Recent advances in the cloning and amplification of human DNA segments in bacterial plasmids... 

Rheumatoid arthritis in association with splenomegaly and neutropenia is known as Felty s syndrome. Thrombocytopenia also may be a manifestation of the syndrome. Patients with Felty s syndrome and severe leukopenia are more susceptible to infection. The decrease in granulocytes appears to be mediated by the immune system because splenectomy does not result in improvement of the patient. ... 

Of the surgical options available prior to enzyme therapy, splenectomy was most often recommended to combat thrombocytopenia and anemia, mechanical complications, and growth retardation. 

The spleen is moderately enlarged in patients with thrombocytopenia, possibly because thrombocytolysis is activated in the spleen. In any event, splenectomy often procures temporary relief or even permanent cure for thrombocytopenia. The various types of thrombocytopenia and other forms of defects in blood coagulation are summarized in Table 7-4. 

In patients undergoing splenectomy, distal or proximal splenic arteries can be completely occluded to reduce the risk of intraoperative hemorrhage. After the embolization, if patients have thrombocytopenia, a femoral arterial closure device may help promote hemostasis at the puncture site. Patients commonly experience fever, leukocytosis, and ele-... 

Few studies have compared splenectomy with PSE in a randomized, prospective fashion. In a series by Mozes et al. [118], 53 patients who were to later undergo renal transplant with azathioprine immunosuppressive therapy (which causes leukopenia and thrombocytopenia) were randomly assigned to a splenectomy group (25 patients) or a PSE group (28 patients). For patients in the PSE group, a mean of... 

In one case splenomegaly was associated with thrombocytopenia and leukopenia which were both reversible with splenectomy. 

Despite the general opinion that gold-induced thrombocytopenia is a self-limiting disorder Herbst et al. have also described 2 patients with persistent thrombocytopenia, finally necessitating splenectomy (48 ). In one of these patients, 18 years after the gold injections, gold was still detectable in the serum and urine, and thrombocytopenia persisted, despite splenectomy. 

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