1/12. Myelofibrosis-associated massive splenomegaly: a cause of increased intra-abdominal pressure, pulmonary hypertension, and positional dyspnea.We describe a patient with myelofibrosis, giant splenomegaly, and pulmonary hypertension related to increased intra-abdominal pressure. Focusing on alterations in hemodynamic studies, we conclude that in patients with myelofibrosis, dyspnea, and hypoxemia, the measurement of intra-abdominal pressure should be included in the initial evaluation. It is an inexpensive, non-invasive diagnostic tool that can provide crucial information about the cause of dyspnea and disclose the pathogenetic link between massive splenomegaly and pulmonary compromise in myelofibrosis.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/12. Diffuse hemangiomatosis of the spleen: splenic hemangiomatosis presenting with giant splenomegaly, anemia, and thrombocytopenia.In an elderly patient with oligosymptomatic giant splenomegaly, clinical and laboratory data were nondiagnostic, while nonhomogeneous splenic enlargement was the only finding detected by imaging procedures. splenectomy was performed and diffuse hemangiomatosis of predominantly capillary-type found. The failure of imaging techniques to even hint at the nature of the underlying disorder is comprehensible in view of the organ being essentially replaced in toto by the abnormal vascular channels. Diffuse splenic hemangiomatosis, a rare condition, may cause hypersplenism, and its diagnosis may be elusive because of misleading patterns on imaging.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
3/12. sarcoidosis and giant splenomegaly.splenomegaly is often a manifestation of sarcoidosis, but giant splenomegaly is rare. Only 25 cases of sarcoidosis presenting as massive splenomegaly have been reported. splenectomy was performed before the diagnosis of sarcoidosis was made in more than half of these patients. Serious complications of giant splenomegaly including rupture, severe thrombocytopenia and hemolytic anemia were not consistently present preoperatively. A case of disseminated sarcoidosis presenting with giant splenomegaly and normal chest roentgenograms is described. prednisone therapy improved the patient's pulmonary function testing while constitutional symptoms, splenomegaly and elevated angiotensin-converting enzyme levels completely resolved. sarcoidosis should be considered in the differential diagnosis of massive splenomegaly. A trial of steroids is warranted as initial treatment, and in selected cases may prevent unnecessary splenectomy, thereby precluding a compromise in host defenses against encapsulated bacteria.- - - - - - - - - - ranking = 7keywords = giant (Clic here for more details about this article) |
4/12. Giant aneurysm of the splenic artery and huge varix.Giant aneurysm of the splenic artery and huge varix accompanied by portal hypertension and splenomegaly are described. Computed tomography proved to be an ideal tool in the evaluation of both aneurysm and varix. Enhancement scan revealed giant aneurysm as a round mass in the splenic hilus and huge varix as one oval and one bell-shaped shadow adjacent to the spleen. They were diagnosed by angiography.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/12. Multicentric giant lymph node hyperplasia clinically simulating angioimmunoblastic lymphadenopathy. Associated Kaposi's sarcoma in two of three cases.We report on three patients whose clinical presentation and laboratory findings were similar to those of angioimmunoblastic lymphadenopathy (AILD). The pathology findings in the lymph nodes were characterized by enlarged lymph follicles, a massive plasma cell infiltration, and marked vascularization. These morphological features were similar to those of giant lymph node hyperplasia, plasma cell type. In two of the patients, there was an associated Kaposi's sarcoma.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
6/12. Partial splenectomy in Gaucher's disease.An 11-year-old boy with Gaucher's disease is described. He had giant splenomegaly with severe signs of hypersplenism. A partial splenectomy was performed. On an isotope scan at follow-up, the remaining spleen was functioning well. Normal levels of IgM were recorded and there were no signs of hypersplenism.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/12. Systemic giant-cell histiocytosis: report of a case and a review of the adult form of Letterer-Siwe disease.The multiple classifications of histiocytic disorders, and the lack of specificity in their application, have degraded the utility of such diagnostic terms as "Letterer-Siwe disease" and "Malignant histiocytosis". A case is presented of a systemic histiocytic proliferation with erythrophagocytosis and giant-cell formation which does not fit into any presently accepted diagnostic niche. No similar case was found in the literature. Moreover, it appears that the entity "adult form of Letterer-Siwe disease" has been uncritically accepted on the basis of a few poorly documented cases, and should be abandoned.- - - - - - - - - - ranking = 5keywords = giant (Clic here for more details about this article) |
8/12. Unusual morphological features of adult T-cell leukemia cells with aberrant immunophenotype.We describe 4 cases of adult T-cell leukemia (ATL) with unusual morphology and aberrant immunophenotype. All patients were Japanese and born in the Nagasaki district, an area endemic for HTLV-I. Peripheral blood and/or bone marrow films revealed bizarre giant cells with and without large nucleoli; the cells were 5 to 6 times the diameter of erythrocytes, resembling Hodgkin's cells. Some peripheral blood cells were morphologically similar to prototypic ATL cells, while many other cells in the bone marrow showed unusual morphology. Furthermore, leukemic cells had aberrant immunophenotypes such as the CD8-positive type in patients 1 and 2, the CD4-.CD8- double-negative type in patient 3, and the CD5 antigen defect in patient 4. All patients had marked elevations of the serum calcium and LDH and organomegaly, while all had a short survival. Anti-htlv-i antibodies and provirus dna monoclonality were demonstrated in all patients. The results suggested that the unusual morphology and aberrant ATL cell immunophenotype may be indicative of a high grade malignant behaviour of ATL.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
9/12. Giant splenomegaly and refractory hypercalcemia due to extrapulmonary sarcoidosis. Successful treatment by splenectomy.sarcoidosis is a granulomatous disease of unknown origin with a variable clinical presentation. The presenting complaint is usually referable to the lung. We describe an unusual presentation of sarcoidosis in a young black man who received medical attention for evaluation of pancytopenia, giant splenomegaly, and marked, refractory hypercalcemia. After extensive evaluation, including exploratory laparotomy, he was found to have sarcoidosis, with extensive involvement of his spleen, liver, and abdominal lymph nodes. Pulmonary involvement was notably absent, with no suggestive findings radiographically on gallium citrate Ga 67 scanning or on bronchoscopy with transbronchial biopsy. This patient underwent splenectomy and, following removal of the massive splenic granuloma burden, the hypercalcemia resolved completely with no other therapy.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/12. Subtotal splenectomy for treatment of patients with myelofibrosis and myeloid metaplasia.Idiopathic myelofibrosis is a chronic myeloproliferative disorder that may course with myeloid metaplasia involving several organs. Occasionally, the spleen reaches giant proportions and must be withdrawn. However, this procedure is followed by greater morbidity and mortality. Partial splenectomies, preserving the splenic pedicle, have been proposed in order to reduce postoperative complications. After an early postoperative improvement, some patients present symptomatic recurrence of their disease. This communication presents a new alternative for the treatment of complicated myeloid metaplasia: subtotal splenectomy, preserving the upper splenic pole supplied only by the splenogastric vessels. We performed this procedure in three patients. Their almost two years postoperative follow-up and health improvement suggest that this operation should be considered for the treatment of huge spleens due to myelofibrosis with myeloid metaplasia.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
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