The disproportionately high rate of overwhelming postsplenectomy infection (OPSI) in thalassemia patients is thought to be due to an immune deficiency. Which of the following strategies has been shown to reduce mortality?
The increase in infectious complications associated with splenectomy in thalassemia patients is thought to be due to a coexisting immune deficiency that is caused by iron overload. Iron overload is associated with both thalassemia as well as the transfusions that accompany treatment for thalassemia. Some investigators have tried partial splenectomy with some success in reducing mortality associated with splenectomy in these patients. In addition, splenectomy should be delayed until the patient is older than 4 years unless absolutely necessary. While transfusion to maintain a hemoglobin (HGB) of >9 mg/dL is part of the treatment for thalassemia it does not reduce infectious complications associated with splenectomy in these patients. There is little evidence supporting efficacy of prophylactic antibiotics in asplenic patients in preventing infectious complications associated with splenectomy.
A 30-year-old woman presents to her primary care provider with complaints of bleeding gums while brushing her teeth as well as menorrhagia and several episodes of epistaxis within the past month. She has been previously healthy with no prior medical problems or surgeries. Examination reveals petechiae and ecchymosis over the lower extremities. Laboratory results show white blood cell (WBC) count 7000/mm3, HGB 14 g/dL, hematocrit (HCT) 42%, and platelet count 28,000/mm3 with numerous megakaryocytes on peripheral smear. First-line therapy for this condition would be:
Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by a low platelet count and mucocutaneous and petechial bleeding. The usual first line of therapy for ITP is oral prednisone with most responses occurring within the first 3 weeks after initiating therapy. Intravenous (IV) immunoglobulin is given for internal bleeding with platelet counts <5000/mm3, when extensive purpura exists, or to increase platelets preoperatively and is thought to work by impairing clearance of immunoglobulin G-coated platelets by competing for binding to tissue macrophage receptors. Both rituximab and thrombopoietin-receptor antagonists are second-line treatment options. Splenectomy is an option for refractory ITP and can provide a permanent response in about 75 to 85% of patients.
The most common physical finding in a patient with hairy cell leukemia (HCL) is:
Hairy cell leukemia (HCL) is an uncommon blood disorder, representing only 2% of all adult leukemias. HCL is characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow. These lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear. Most patients seek medical attention because of symptoms related to anemia, neutropenia, thrombocytopenia, or splenomegaly. The most common physical finding is splenomegaly, which occurs in 80% of patients with HCL and the spleen is often palpable 5 em below the costal margin. Many patients with HCL have few symptoms and require no specific therapy. Treatment is indicated for those with moderate to severe symptoms related to cytopenias, such as repeated infections or bleeding episodes, or to splenomegaly, such as pain or early satiety. Splenectomy does not correct the underlying disorder, but does return cell counts to normal in 40 to 70% of patients and alleviates pain and early satiety. Newer chemotherapeutic agents (the purine analogues 2' -deoxycoformycin [2' -DCF] and 2-chlorodeoxyadenosine [2-CdA] ) are able to induce durable complete remission in most patients.
Which of the following is an indication for splenectomy in a patient with chronic myelogenous leukemia ( CML)?
Chronic myelogenous leukemia (CML) is a disorder of the primitive pluripotent stem cells in the bone marrow, resulting in a significant increase in erythroid, megakaryotic, and pluripotent progenitors in the peripheral blood smear. The genetic hallmark is a transposition between the bcr gene on chromosome 9 and the abl gene on chromosome 22. CML accounts for 7 to 15% of all leukemias, with an incidence of 1.5 in 100,000 in the United States. CML is frequently asymptomatic in the chronic phase, but symptomatic patients often present with the gradual onset of fatigue, anorexia, sweating, and left upper quadrant pain and early satiety secondary to splenomegaly. Enlargement of the spleen is found in roughly one-half of patients with CML. Splenectomy is indicated to ease pain and early satiety.
Which of the following is an indication for splenectomy in polycythemia vera (PV)?
Polycythemia vera (PV) is a clonal, chronic, progressive myeloproliferative disorder characterized by an increase in RBC mass, frequently accompanied by leukocytosis, thrombocytosis, and splenomegaly. Patients affected by PV typically enjoy prolonged survival compared to others affected by hematologic malignancies, but remain at risk for transformation to myelofibrosis or acute myeloid leukemia (AML). The disease is rare, with an annual incidence of 5 to 17 cases per million population. Although the diagnosis may be discovered by routine screening laboratory tests in asymptomatic individuals, affected patients may present with any number of nonspecific complaints, including headache, dizziness, weakness, pruritus, visual disturbances, excessive sweating, joint symptoms, and weight loss. Physical findings include ruddy cyanosis, conjunctival plethora, hepatomegaly, splenomegaly, and hypertension. The diagnosis is established by an elevated RBC mass (>25% of mean predicted value), thrombocytosis, leukocytosis, normal arterial oxygen saturation in the presence of increased RBC mass, splenomegaly, low serum erythropoietin (EPO) stores, and bone marrow hypercellularity. Treatment should be tailored to the risk status of the patient and ranges from phlebotomy and aspirin to chemotherapeutic agents. As in essential thrombocythemia (ET) splenectomy is not helpful in the early stages of disease and is best reserved for late-stage patients in whom myeloid metaplasia has developed and splenomegaly-related symptoms are severe.
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