Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 79-year-old male was admitted to our hospital because of general fatigue and night sweat. Physical examination showed generalized superficial lymphadenopathy, marked splenomegaly, and tumors in the conjunctiva and the abdomen. Chest X-ray and computed tomography (CT) revealed pleural effusion and intrathoracic lymphadenopathy. Abdominal ultrasonography and CT showed hepatosplenomegaly and intraperitoneal tumors. Upper gastrointestinal fiberscopy revealed multiple polypoid lesions and ulcers in the duodenum and the stomach. Involvement of relatively small-sized lymphocytes with cleaved nuclei was identified in each biopsied specimen from a cervical lymph node, a tumor in the conjunctiva, gastrointestinal polypoid lesions, and the bone marrow. Surface marker analysis of abnormal lymphocytes in the bone marrow revealed that CD5, CD19, and CD20 were strongly positive, but CD23 was weakly positive. Although (11:14)(q13:q32) translocation was not identified by chromosome analysis of bone marrow cells, Northern blot analysis of bone marrow cells revealed overexpression of the PRAD1 oncogene. Diagnosis of mantle cell lymphoma (MCL) was made. Combination chemotherapy by cyclophosphamide and vincristine was not effective, but etoposide perorally given at a dose of 50 mg per day was effective. In MCL, extranodal involvement of a digestive tract and bone marrow is well known. This case suggests that involvement of multiple organs including lacrimal glands and pleura could be characteristic of MCL cells.
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PMID:[Mantle cell lymphoma with multiple extranodal involvement]. 924 28

An indolent variant of splenic marginal zone lymphoma (SMZL) lacking massive splenomegaly has been described as an incidental finding in spleens removed for rupture or hypersplenism. We studied traumatically ruptured spleens with expanded marginal zones (MZs) to assess the incidence of occult monoclonal B-cell populations in this setting. Ninety-one ruptured or lacerated spleens removed from 1984 to 1995 were classified as to whether they had expanded MZs (> 12 cell layers thick). When available, paraffin-embedded, formalin-fixed tissue from cases with expanded MZs was examined for immunoglobulin heavy chain gene rearrangement by polymerase chain reaction (PCR) and stained for CD20, CD43, and kappa and lambda light chains. Splenectomies were performed for blunt (70 patients) and penetrating (7 patients) trauma, surgical misadventure (13 patients), or spontaneous rupture (1 patient). There were 58 men and 33 women in our study, ranging in age from 17 to 87 years (mean, 40 yr). Average spleen weight was 183 g (range, 44-505 g). Twenty-seven (30%) of 91 patients had expanded MZs. There were no significant differences in age, sex, spleen weight, or reason for excision between those cases with and without MZ expansion. Germinal centers varied from absent to inactive to floridly reactive. Paraffin blocks were available in 24 cases; the 20 with amplifiable DNA were polyclonal by PCR. Follow-up was available for 25 of the 27 patients with expanded MZs (range, 1-85 mo; median, 6 mo); lymphoma did not develop in anyone, although one patient's spleen was morphologically suspicious for lymphoma, showing involvement of red pulp by MZ-type B-cells; PCR revealed a polyclonal pattern. This patient's 3-year follow-up revealed no evidence of lymphoma. Traumatically ruptured spleens with expanded MZs do not seem to harbor occult B-cell clones, as detected by PCR. Although a few cases of incidentally removed spleens have been reported to contain low-stage SMZL, this seems to be an infrequent event.
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PMID:Ruptured spleens with expanded marginal zones do not reveal occult B-cell clones. 943 66

We report the case of a 78-year-old man in whom routine physical examination revealed cervical adenopathy and splenomegaly. Peripheral blood showed a normal white blood cell count with an absolute lymphocytosis, which included a population with slightly indented nuclei. Lymph node biopsy showed morphology compatible with mantle cell lymphoma. Bone marrow biopsy showed replacement by a lymphoid proliferation composed of lymphocytes with features similar to those found in the peripheral blood. Immunophenotypic analysis of both peripheral blood and lymph node showed positivity for CD19, CD20 and CD22, with lambda light chain restriction. Tests for CD5 and CD10 were negative. Cytogenetic analysis and polymerase chain reaction studies confirmed the presence of t(11,14) supporting a diagnosis of mantle cell lymphoma. This unusual case of CD5-negative mantle cell lymphoma exemplifies the importance of combined molecular, cytogenetic, and morphologic evaluation when confronted with a lymphoma having an atypical phenotype.
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PMID:CD5 negative diffuse mantle cell lymphoma with splenomegaly and bone marrow involvement. 963 25

We describe a case of B-cell prolymphocytic leukemia (PLL) successfully treated with splenic irradiation (SI). A 69-year-old woman was admitted to our hospital because of massive splenomegaly and leukocytosis. Peripheral blood showed hemoglobin (Hb) 7.4 g/dl, platelets 48 x 10(9)/l and white blood cells (WBC) 50.3 x 10(9)/l with 90% prolymphocytes. Bone marrow was hypercellular with 60% prolymphocytes. Surface marker analysis revealed that prolymphocytes were positive for CD20, CD22, FMC7, HLA-DR and surface immunoglobulin (mu, delta and lambda), but negative for CD5 and mouse erythrocyte rosette. A diagnosis of B-cell PLL was made. SI (1.5 Gy x 4/week, total dose 19.5 Gy) was chosen for the treatment and a remarkable response was achieved immediately after the first irradiation. Finally, a single course of SI induced complete remission without any significant side effect. One year after the SI, she showed no splenomegaly and almost normal peripheral blood cell count (Hb 11.2 g/dl, platelets 100 x 10(9)/l, WBC 3.6 x 10(9)/l with 71% neutrophils and no prolymphocyte). She has been well for more than 24 months. This case showed that SI may remain valuable for an initial course of PLL treatment.
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PMID:Splenic irradiation for prolymphocytic leukemia: is it preferable as an initial treatment or not? 965 13

We report a case of mantle cell lymphoma in leukemic phase, which was diagnosed by a bone marrow biopsy performed as part of a workup for chronic anemia in a patient without lymphadenopathy. The patient, a 79-year-old man with diabetes mellitus, hypertension, chronic renal failure, congestive heart failure, and atherosclerosis, presented with claudication. On admission, he also had an 8-month history of anemia, during which time he experienced a 18-kg weight loss. On presentation, the patient had normal vital signs, anemia, leukocytosis (as well as an absolute lymphocytosis), and splenomegaly; as mentioned, lymphadenopathy was absent. A bone marrow biopsy showed an increase in small to intermediate-sized, slightly irregular lymphocytes in interstitial nodules. Flow cytometric immunophenotyping of the bone marrow identified a monoclonal population of cells, representing 25% of cells within the bone marrow, with expression of CD19, CD20, immunoglobulin M/D, lambda light chain, HLA-DR, and CD5; reactions for CD10 and CD23 were absent. Based on morphologic and immunophenotypic analysis of the bone marrow, as well as morphologic review of the peripheral blood smear, a diagnosis of mantle cell lymphoma involving the bone marrow and in leukemic phase was made. Subsequent polymerase chain reaction analysis of DNA from peripheral blood identified a population of cells with the bcl-1 rearrangement. This case is unique in that the diagnosis of mantle cell lymphoma was made without lymph node or spleen analysis and the patient, although exhibiting bone marrow and peripheral blood involvement by mantle cell lymphoma at presentation, did not have lymphadenopathy.
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PMID:Leukemic phase of mantle cell lymphoma presenting as anemia: diagnosis by combining flow cytometry and cytomorphology. 982 32

B-cell lymphoma associated with haemophagocytic syndrome (HPS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries. We describe seven patients with B-cell lymphoma associated with HPS, six males and one female, age range 41-82 years (median 63 years). All patients had fever and splenomegaly, and six of the seven patients had hepatomegaly with no associated lymphadenopathy. The bone marrow showed haemophagocytosis and an infiltration of lymphoma cells. All patients showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Lymphoma cells were positive for CD19. CD20 and surface immunoglobulin in all patients examined, and positive for CD5 in four of seven patients. Cytogenetic analyses of bone marrow cells showed a complex structural abnormality including chromosome 14q32 in two patients, 19q13 in three patients and deletion of the terminal part of 8p21 in six patients. The prognosis was poor; only two of the seven patients have survived in complete remission with a median survival of 11 months. These data suggested that B-cell lymphoma associated with HPS might constitute a distinct biological and clinical disease entity. Abnormality of chromosome 19q13 and loss of 8p21 might be involved in the pathogenesis of this disease.
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PMID:B-cell lymphoma associated with haemophagocytic syndrome: a clinical, immunological and cytogenetic study. 1052 18

An 88-year-old Japanese woman with splenomegaly, but without lymphadenopathy, was admitted because of epigastric distress. Laboratory data disclosed an RBC of 310 x 10(4)/microliter, Hb of 10.1 g/dl, Ht of 30.6%, Plt count of 9.8 x 10(4)/microliter, and WBC of 4,470/microliter with 38% abnormal lymphocytes. Peripheral blood films revealed lymphocytes with thin, short cytoplasmic villi, condensed nuclear chromatin, and small nucleoli. The lymphocytes stained negative for tartrate-resistant acid phosphatase. Also, immunophenotyping was positive for expression of the cell surface markers CD19, CD20, IgG, kappa and HLA-DR, but not for CD5, CD10, CD11c, CD23, CD25, CD38, or CD103 antigens. Chromosomal analysis of peripheral blood cells disclosed the 46, XX, del(7), (q32) aberration. A splenectomy was performed simultaneously with partial colon resection because of a mucinous carcinoma found in the transverse colon. Histologic examination of resected spleen tissues revealed a distinctive pattern of white pulp infiltration by lymphoma cells. The histologic findings and clinical data were consistent with the features of splenic lymphoma with circulating villous lymphocytes. Our patient exhibited a relatively benign clinical course, and was being followed on an outpatient basis with no additional therapy.
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PMID:[Splenic lymphoma with villous lymphocytes expressing chromosomal abnormalities]. 1035 43

A case of intestinal angiocentric T/NK-cell lymphoma in a 58-year-old man is reported. The patient presented initially with panperitonitis because of perforation of sigmoid colon diverticulum. He underwent segmentectomy of involved bowel. Histologically, the intestinal wall showed diffuse infiltration of medium or large size lymphoma cells with angiocentric growth and necrosis. The lymphoma cells were CD56+, CD45RO+, CD3+, CD4-, CD8-, CD20-, and CD30- in paraffin sections with germline configuration of TCR-gamma gene, consistent with T/NK-cell lymphoma. Further staging revealed splenomegaly. Intestinal angiocentric T/NK cell lymphoma represents a distinct etiology of diverticulum with perforation.
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PMID:Extranasal T/NK-cell lymphoma presenting as intestinal diverticulum. 1080 3

B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) is extremely rare in Western countries but has recently been increasingly reported in Asian countries, especially Japan. Here, we reviewed 25 previously reported Japanese cases of B-LAHS and summarized its clinicopathologic features and therapeutic outcome. The median age of onset was 63 years old with initial presentation of fever, hepatomegaly, and splenomegaly without associated lymphadenopathy. Laboratory findings showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. Histopathologically, hemophagocytosis was often seen in the bone marrow and spleen. Various percentages of lymphoma cells were seen in the bone marrow, positive for CD19, CD20 and surface immunoglobulin. and some were also positive for CD5. Cytogenetic analysis showed a complex structural abnormality including chromosome 14q32, 19q13 and deletion of the terminal part of 8p21. Some patients had histological features of intravascular lymphomatosis (IVL). The prognosis was poor with a median survival period of 9 months. We treated five patients using autologous peripheral blood stem cell transplantation (PBSCT), and four are still in complete remission nine to 24 months after PBSCT, suggesting that high-dose chemotherapy followed by PBSCT might improve the survival rate.
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PMID:B-cell lymphoma-associated hemophagocytic syndrome. 1081 54

We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenstrom's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.
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PMID:Splenic marginal zone lymphoma with or without plasmacytic differentiation. 1111 78


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