spleen wbc disorders

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White Blood Cell DisordersThe role of splenectomy in patients with white blood cell disorders. Splenectomy for white blood cell disorders can be effective therapy for symptomatic splenomegaly and hypersplenism, improving some clinical parameters but generally not altering the course of the underlying disease. Careful consideration of the intended benefits of splenectomy must be weighed against the significant perioperative and postsplenectomy risks in this often complex patient population.Hairy Cell Leukemia Uncommon blood disorder, 2% of all adult leukemias. Characterized by splenomegaly, pancytopenia, and large numbers of abnormal lymphocytes in the bone marrow. Lymphocytes contain irregular hair-like cytoplasmic projections identifiable on the peripheral smear. Have few symptoms and require no specific therapy Splenectomy does not correct the underlying disorder but does return cell counts to normal in 40% to 70% of patients and alleviates symptoms of splenomegaly. Advent of diverse new drugs (e.g., rituximab, pentostatin, cladribine), splenectomy has become rarely indicated.Hodgkins Disease Disorder of the lymphoid system characterized by the presence of Reed-Sternberg cells 90% of patients with HD present with lymphadenopathy above the diaphragm. Lymph nodes can become particularly bulky in the mediastinum, which may result in shortness of breath, cough, or obstructive pneumonia. Lymphadenopathy below the diaphragm is rare on presentation but can arise with disease progression. The spleen is often an occult site of spread, but massive splenomegaly is not common. Four major histologic types exist: lymphocyte predominance type, nodular sclerosis type, mixed cellularity type, and lymphocyte depletion type. The histologic type, along with location of disease and symptomatology, influence survival for patients with HD. Stage I disease is limited to one anatomic region; Stage II disease is defined by the presence of two or more contiguous or noncontiguous regions on the same side of the diaphragm; Stage III disease involves disease on both sides of the diaphragm, but limited to lymph nodes, spleen, and Waldeyers ring (the ring of lymphoid tissue formed by the lingual, palatine, and nasopharyngeal tonsils); Stage IV disease includes involvement of the bone marrow, lung, liver, skin, gastrointestinal tract, or any organ or tissue other than the lymph nodes or Waldeyers ring. Current indications for surgical staging include clinical suspicion of lymphoma without evidence of peripheral disease or patients requiring restaging for suspicion of failure after chemotherapy.Non-Hodgkins Lymphoma Proliferation of any one of the three predominant lymph cell typesnatural killer cells, T cells, or B cellsmay be included in the category of NHL Clinical presentations of the disorder under its umbrella vary. Subentities of NHL may be clinically classified into nodal or extranodal, as well as indolent, aggressive, and very aggressive groups. Patients with indolent lymphomas may present with mild or no symptoms and seek medical attention for a swollen lymph node, whereas the aggressive and very aggressive lymphomas create easily noticeable symptoms, such as pain, swelling due to obstruction of vessels, fever, and night sweats. Surgical staging is no longer indicated because the combination of history and physical examination, chest radiograph and abdominal/pelvic CT scan, biopsy of involved lymph nodes (including laparoscopically directed nodal and liver biopsies), and bone marrow biopsy is sufficient. Splenomegaly exists in some, but not all, forms of NHL. Splenectomy is indicated in cases where a diagnosis cannot be established by obtaining peripheral tissue and clinical suspicion remains or for management of symptoms related to an enlarged spleen as well as for improvement of cytopenias. Splenectomy does not alter the natural history of the disease, but related thrombocytopenia may improve in up to 75% of patients. Chronic Lymphocytic Leukemia Considered a subtype of NHL The main characteristic is a progressive accumulation of old and nonfunctional lymphocytes. Symptoms are nonspecific and include weakness, fatigue, fever without illness, night sweats, and frequent bacterial and viral infections. The most frequent finding is lymphadenopathy. When the spleen is enlarged, it may be massive or barely palpable below the costal margin. Splenectomy is indicated to improve cytopenias and was shown to be 75% effective in a combined group of patients who had either CLL or nonmalignant HD Splenectomy may facilitate chemotherapy in patients whose cell counts were prohibitively low before spleen removal. Palliative splenectomy also is indicated for symptomatic splenomegaly.