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

We report two cases of a T cell lymphoproliferative disease not previously described, with cytologic and clinical features similar to those associated with Galton's "prolymphocytic" leukemia (PL). Our patients, like those with Galton's PL, had massive splenomegaly and minimal or absent hepatomegaly and lymphadenopathy. In contrast, however, our patients had leukopenia, as well as low percentages of leukemic cells in the peripheral blood and in the bone marrow. In splenic imprints, the nuclear chromatin pattern of most of the leukemic cells was intermediate between those of mature lymphocytes and those of lymphoblasts, and the nuclei contained single, centrally located, conspicuous nucleoli. In sections of the spleen, the leukemic cells diffusely infiltrated the red pulp in a pattern strikingly similar to that of hairy cell leukemia; however, when the leukemic cells were studied cytochemically, the cytoplasmic acid phosphatase positivity was punctate and tartrate-sensitive. The leukemic cells were sheep erythrocyte rosette-positive and expressed T cell-associated antigens. Initially, both patients responded well to therapeutic splenectomy. One patient received combination chemotherapy after splenectomy and is alive and well 24 months after diagnosis. The other patient was in complete clinical remission for one year after splenectomy and received chemotherapy at relapse. He died, however, 23 months after splenectomy, with disseminated disease. IgG antibody titers against human T lymphotropic virus type I (HTLV-I) were detected in one patient and against HTLV-II in the other. The leukemia in these patients represents a distinct clinicopathologic entity within the spectrum of peripheral T cell lymphoproliferative diseases that includes Galton's PL of T cell derivation, T cell chronic lymphocytic leukemia, T cell hairy cell leukemia, and adult T cell leukemia/lymphoma.
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PMID:Leukopenic chronic T cell leukemia mimicking hairy cell leukemia: association with human retroviruses. 300 37

A 68-year-old Japanese man with a chief complaint of eczema-like dermatosis was diagnosed as having B-cell hairy cell leukemia (HCL) by demonstration of hairy cells in the skin lesions as well as in blood and bone marrow. He was treated with alpha-interferon, resulting in disappearance of skin lesions and reduction of his massive splenomegaly from 18 to 5 cm in about 14 months. Although specific skin lesions in HCL, shown by a review of the literature to occur in about 8% of cases, are not as uncommon as generally assumed, it is rare for HCL to present with specific skin lesions, the present case being only the second of its type mentioned in the literature.
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PMID:Specific skin lesions as the presenting symptom of hairy cell leukemia. 305 17

Hairy cell leukemia is a rare lymphoproliferative disorder which is morphologically characterized by circulating lymphatic cells with prominent 'hairy' cytoplasmatic projections. Patients typically present with pancytopenia and splenomegaly. The bone marrow cannot be aspirated because of reticulin fibrosis. Chemotherapy was unable to influence the course of the disease satisfactorily. Therefore, splenectomy was the treatment of choice. The introduction of alpha-interferons into the therapy of hairy cell leukemia was a decisive breakthrough and is exemplary for the use of interferons in the treatment of malignant diseases. However, there are still many questions to be answered. The discussion of clinical implications is achieving growing importance in consequence of the availability of diverse commercial alpha-interferons for treatment of hairy cell leukemia.
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PMID:[Therapy of hairy cell leukemia with interferon]. 305 67

HCL is a well-recognized entity among the lymphoproliferative disorders. With better appreciation of the wide variability in its clinical and hematologic manifestations, some authors have proposed several subtypes of HCL such as leukopenic and nonleukopenic subtypes and subtypes with and without massive splenomegaly. As opposed to such a clinical and hematologic variability, the pathology of HCL in the spleen and bone marrow is consistent and highly characteristic. Since the spleen becomes available for pathologic examination only after therapeutic splenectomy, the bone marrow pathology often plays the most important role in the differential diagnosis of HCL. It is characterized by focal or diffuse mononuclear cell infiltration with a wide spacing between individual nuclei in most patients and by a severely hypocellular marrow with individual hairy cells infiltrating between the marrow fat cells in the remaining minority of patients. The bone marrow biopsy also serves as one of the criteria in selecting the therapeutic modality as well as monitoring the therapeutic effect. Further, new insights into the pathogenesis of HCL are emerging from recent studies of the bone marrow microenvironment.
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PMID:Bone marrow in hairy cell leukemia. 306 20

Hairy cell leukemia is a chronic lymphoproliferative disorder characterized clinically by splenomegaly and cytopenias. Spontaneous remissions are rare and splenectomy is often performed when the blood counts worsen and cause symptoms. Three of our patients with hairy cell leukemia developed recurrent pancytopenia and transfusion-dependent anemia after splenectomy. Each subsequently acquired transfusion hepatitis and in two patients marked hematologic improvement was noted within 2 months. Complete peripheral blood remission occurred within 17 months in all patients although bone marrow infiltration with hairy cells persisted. One patient remains in remission for 12 years; the other two succumbed to infectious illnesses but with normal blood counts. The mechanism by which hepatitis virus induces hematologic recovery in patients with hairy cell leukemia is unknown but may involve augmentation of the interferon system.
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PMID:Peripheral blood remission of hairy cell leukemia after transfusion hepatitis. 311 Dec 50

Ten nonsplenectomized patients (5 males, 5 females) with hairy-cell leukemia were treated with recombinant alpha-IFN. The median age of patients was 54 years (32-76 years). The time between diagnosis and onset of treatment ranged from 1-120 months. Initially the patients received 5 x 10(6) I.U. daily by intramuscular or subcutaneous injections for 10 weeks. Thereafter the dose of IFN was reduced to 5 x 10(6) I.U. 3 x weekly and after 6 months to 5 x 10(6) I.U. 2 x weekly for at least 6 months. The median time of patient follow-up is 23 months (4-28 months). Three complete remissions were achieved after 3-24 months and five partial remissions after 4-18 months. One patient showed improvement after 3 months, and one patient died from cerebral hemorrhage after 5 weeks. In eight of ten patients peripheral blood cell counts normalized after a median of 7 months (1.5-11 months). A marked reduction in spleen size was observed in all seven patients who had splenomegaly at study entry. Two of three patients who interrupted therapy have relapsed after 2 and 6 months. We conclude that alpha-interferon is an effective method of first-line treatment in patients with HCL.
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PMID:Recombinant alpha-interferon (alpha-IFN) for progressive hairy-cell leukemia (HCL): a study in previously untreated nonsplenectomized patients. 334 41

Two patients with hairy cell leukemia with massive splenomegaly and severe pancytopenia were treated with recombinant alpha-A interferon (IFN-alpha-2a). There was no significant response to a trial of IFN-alpha-2a (11 and 20 weeks) with respect to blood counts or spleen size. Subsequent treatment with 2'-deoxycoformycin (dCF) for 8 consecutive weeks (4 mg/m2/wk) resulted in normalization of spleen size and a normalization of peripheral blood counts and bone marrow in one patient. The second patient demonstrated a reduction in spleen size and improved blood counts following 9 weeks of dCF therapy but eventually became refractory. This demonstrates that dCF is non-cross-resistant with interferon and confirms the efficacy of dCF in nonsplenectomized patients.
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PMID:Response to 2'-deoxycoformycin after failure of interferon-alpha in nonsplenectomized patients with hairy cell leukemia. 348 58

A 47-year-old man, was referred for evaluation of asymptomatic splenomegaly in September 1981, and a diagnosis of hairy cell leukemia (HCL) at the initial clinical stage was made. The patient remained asymptomatic until May 1985, when splenectomy was performed because of anemia and splenomegaly. Bone marrow and liver biopsy specimens showed diffuse infiltration by abnormal tartase resistant acid phosphatase (TRAP) positive lymphocytes with typical aspect of hairy cells. Four months later, he developed fever of unknown origin and, at laparotomy, diffuse retroperitoneal lymph node enlargement and metastatic liver nodules were seen. Lymph node and liver biopsy specimens showed diffuse infiltration by abnormal large lymphocytes, which bore monoclonal surface immunoglobulin M and light chain kappa. Only six cases of non-Hodgkin's lymphoma associated with HCL have been published to date. This report describes an additional case of immunoblastic B-cell lymphoma, preceded 4 years earlier by the diagnosis of HCL.
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PMID:Occurrence of immunoblastic B-cell lymphoma in hairy cell leukemia. 349 61

The clinical and laboratory features of 23 patients with a characteristic form of splenic lymphoma with circulating "villous" lymphocytes (SLVL) are described and compared with those of other B cell disorders with preferential splenic involvement. SLVL affects predominantly men in their early 70's and is characterized by gross splenomegaly with little or no lymphadenopathy, presence of monoclonal gammopathy in two thirds of the cases, and infiltration of the peripheral blood by lymphocytes with a characteristic pattern of membrane irregularity. These lymphocytes are often confused with cells from hairy cell leukemia, from which they can be distinguished by a number of morphological features, by having a small cell volume, and by lack of expression of the HC2 and Tac antigens. The bone marrow is easily aspirated in the majority of cases and shows a relatively sparse infiltration. The spleen histology shows predominant white pulp involvement, although infiltration of the red pulp is seen in a small number of cases. The differential diagnosis of SLVL should also include hairy cell leukemia variant, prolymphocytic leukemia, and atypical forms of chronic lymphocytic leukemia.
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PMID:Splenic B cell lymphoma with "villous" lymphocytes in the peripheral blood: a disorder distinct from hairy cell leukemia. 349 40

Twelve evaluable patients with progressive hairy cell leukemia were treated with deoxycoformycin at a dose of 4 mg/m2 every 2 weeks. Five patients had not been splenectomized, and one had failed to respond to interferon-alpha. Complete remission, as defined by absence of hairy cells in the bone marrow and normalization of the peripheral blood and regression of splenomegaly, was obtained in 11 of 12 patients (92%). These patients have remained in unmaintained remission for 1+ to 13 months with an average of 7.5 months. Two of these patients had a bone marrow relapse at 8 and 12 months, respectively. During treatment the monocytopenia corrected, and, after complete remission was obtained, marrow was aspirable. Toxicity was mild and reversible. There were no significant infections associated with this treatment. It was of interest that we could treat two patients with creatinine clearance of 50 and 60 ml/min using lower doses (and 2-3 mg/m2) than our conventional therapy of 4 mg/m2 every 2 weeks. They obtained a complete remission after 6 and 10 treatments, respectively. Low-dose deoxycoformycin has proven to be an excellent treatment for hairy cell leukemia.
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PMID:Treatment of hairy cell leukemia: the Ohio State University experience with deoxycoformycin. 349 42


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