Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A Mexican-American boy presented at birth with an extensive eruption consisting of 0.5 to 1.0 cm hyperpigmented macules with a distinct peripheral scale involving primarily the forearms, abdomen and lower back (Fig. 1). Rare intact vesicopustules were also identified. There was an unremarkable prenatal history, and the infant was a product of a normal vaginal delivery. With the exception of the skin lesions and moderate hepatosplenomegaly, the physical examination was normal. Gram stains of the pustules showed numerous neutrophils but no bacteria. Bacterial cultures, of the skin and blood, TORCH screen (toxoplasmosis, rubella, cytomegalic virus, and herpes virus) and a VDRL were negative. On the second day of life, the patient developed several pustules with surrounding erythema consistent with erythema toxicum neonatorum. Wright-stained smears of these lesions showed abundant eosinophils. Hepatosplenomegaly resolved by the third day of life and at the time of discharge only hyperpigmented macules persisted. Follow-up visit six weeks later showed no evidence of skin lesions.
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PMID:Transient neonatal pustular melanosis. 51 27

It has been known that tsutsugamushi disease, so-called "Shichito-fever", is widely spread among the Izu Islands, Tokyo Metropolis. The cases were reported in Oshima Island, Niijima Island, Shikine Island, Kozu Island, Miyake Island, Mikura Island, and Hachijo Island previously, although no case has been reported in Toshima Island. In this paper, we report the first two cases of tsutsugamushi disease found in Toshima Island in December 1988 and December 1989. The first case was a 73 year-old male and the second case was a 83 year-old female, respectively. Fever, erythema and eschar were observed in both cases, while lymphadenopathy and hepatosplenomegaly were not detected. After tetracycline was administered, the fever immediately went down and erythema gradually disappeared in both cases. Specific immunofluorescence tests demonstrated that IgG antibody titers rose against Karp, Gilliam and Kato strains, and that IgM antibody titers rose only against Gilliam strain in both cases. Therefore, Gilliam-like strain of Rickettsia tsutsugamushi may play an important role in Toshima Island.
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PMID:[The first two cases of tsutsugamushi disease found in Toshima Island, Tokyo Metropolis]. 190 85

Streptobacillus moniliformis is an uncommon human pathogen contracted from exposure to rodents. It usually produces a mild, protracted illness (rat-bite fever, Haverhill fever, erythema arthriticum epidemicum) that has either a favorable response to antibiotic therapy or spontaneously resolves. This report describes a fatal case of Streptobacillus moniliformis in an infant bitten by a wild rat. The autopsy findings included an interstitial pneumonia, fibrinous endocarditis, mild mononuclear meningitis, hepatosplenomegaly and lymphadenopathy, erythrophagocytosis, and sinusoidal mononuclear cell infiltrates in regional lymph nodes and the liver. To the authors' knowledge, this is the first report of the autopsy pathology findings of this agent.
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PMID:Fatal Streptobacillus moniliformis infection in a two-month-old infant. 271 62

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly, but flared up after several years. Skin lesions appeared in the form of erythema, papules, or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was 0%-2%. The serum lactate dehydrogenase (LDH) value was within normal range and was not associated with hypercalcemia; lymphadenopathy, hepatosplenomegaly, and bone marrow infiltration were very slight. In most cases, hypergammaglobulinemia was seen, and in one case, monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their anti-ATLA antibodies were positive; none had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness and died of cryptococcus meningitis. Based on clinical and pathologic differences, we believe that these cases should be distinguished from typical ATL cases for the purposes of prognosis and treatment.
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PMID:A proposal for smoldering adult T-cell leukemia: a clinicopathologic study of five cases. 622 22

We have observed five patients with smoldering adult T-cell leukemia (ATL) who had skin lesions as premonitory symptoms. The illness developed slowly but flared up after several years. Skin lesions appeared in the form of erythema, papules or nodules. Infiltration of the skin by ATL cells was slight, and the proportion of ATL cells in the peripheral blood was from 0% to 2%. The serum lactic dehydrogenase value was within normal range, and was not associated with hypercalcemia, lymphadenopathy, or hepatosplenomegaly, and bone marrow infiltration was very slight. In most cases, hypergammaglobulinemia was seen, and in one case monoclonal hypergammaglobulinemia was observed. All five patients had lived in an area in which ATL was endemic, and their sera were positive for anti-ATL-associated antigen antibodies. None of them had ever received a blood transfusion. One patient developed typical ATL after more than 13 yr of illness, and died of renal insufficiency. Another patient developed typical ATL after 5 yr of illness, and died or cryptococcus meningitis. These cases were clinically and pathologically different from typical ATL cases already reported, and we feel it necessary to make distinctions from the viewpoints of prognosis and treatment. In discussing these cases, we compared smoldering ATL with typical ATL, and deliberated upon the causes of both.
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PMID:A proposal for smoldering adult T-cell leukemia--diversity in clinical pictures of adult T-cell leukemia--. 660 27

We present an 18-year-old woman who was diagnosed with acute myeloblastic leukemia (AML M2), and in whom chromosome analysis of bone marrow cells revealed t(7;11), an abnormality rarely found in leukemias with a differentiation potency. She relapsed 1 year after complete remission was achieved by chemotherapy. Bone marrow examination then revealed a t(7;11) abnormality in 48 of 50 metaphases examined, even when there were less than 7.5% leukemic blasts in the marrow, indicating that the morphologically normal cells were derived from leukemic blasts. The number of leukemia clones with the additional abnormalities in chromosome 5 increased, with concurrent development of eosinophilia, fever, asthma-like symptoms, erythema, itching, and hepatosplenomegaly. Elevation of interleukin 5 (IL-5) in serum and an enhanced expression of IL-5 mRNA were also detected. The increase in IL-5 may have been produced by an abnormality on chromosome 5.
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PMID:Acute myeloblastic leukemia (M2) with translocation (7;11) followed by marked eosinophilia and additional abnormalities of chromosome 5. 765 2

A five-month-old girl developed high fever, erythema, hepatosplenomegaly and generalized lymphadenopathy. Laboratory examinations revealed elevated peripheral leukocyte counts, C-reactive protein, lactate dehydrogenase and serum ferritin level. Pathologic examination of the lymph nodes revealed immunoblastic lymphadenopathy (IBL) on the basis of the complete effacement of the normal architecture, replacement by a diffuse infiltrate composed of immunoblasts, plasmacytoid cells and small lymphocytes, and an abortive proliferation of blood vessels. B-cells and T-cells were nearly equally mixed throughout the lymph nodes. No rearrangements of the B-cell immunoglobulin and T-cell receptor genes were detected. The patient was initially treated with alpha-interferon with dramatic efficacy. After relapse, however, the disease was well controlled with cyclosporin A (CyA) and subsequently with combination regimens of CyA, deoxyspagarin and azathioprine with fair success. An alternating regimen of 6-mercaptopurine, cyclophosphamide and methotrexate was then instituted which continued the complete remission for 12 months. The effects of immunosuppressants in the treatment of IBL merit investigation.
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PMID:Immunoblastic lymphadenopathy in a five-month-old girl: successful treatment with immunosuppressants. 807 3

POEMS syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of POEMS syndrome as a paraneoplastic syndrome in myeloma patients.
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PMID:POEMS syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma. 1032 21

PRESENTING FEATURES: An 18-year-old white man was admitted to the Osler Medical Service with the chief complaint of back pain. Two weeks prior to admission, the patient developed diffuse and aching upper back pain. Over the next couple of days, he also developed severe anterior chest pain that was somewhat pleuritic in nature but diffuse and extending bilaterally into the shoulders. One week prior to admission, he developed intermittent fevers and night sweats. The patient denied any lymphadenopathy, pharyngitis, sick contacts, shortness of breath, rash, or bleeding. He was seen by a physician and told that he had thrombocytopenia. There was no history of recent or remote unusual bleeding episodes. His medical history was unremarkable except for a childhood diagnosis of attention deficit/hyperactivity disorder. He was not taking any medications and had no history of tobacco, alcohol, or illicit drug use. He had no risk factors for human immunodeficiency virus infection. Physical examination showed that he was afebrile and had normal vital signs. He was a well-appearing man who was lying still because of pain. HEENT examination was unremarkable. There was no pharyngeal erythema or exudates. His lungs were clear. His neck was supple and without lymphadenopathy. Examination of his back and chest revealed no focal tenderness. There was no hepatosplenomegaly, and his skin was without petechiae or rashes. Examination of the patient's joints showed pain on passive and active movement of his shoulders bilaterally, but no frank arthritis. There was no rash, petechiae, or echymoses. Chest radiograph and electrocardiogram were unremarkable. On admission, the laboratory examination was notable for a hematocrit level of 32.5%, with a mean corpuscular volume of 79 fL, and white blood cell count of 2.8 x 10(3)/microL. Platelet count was 75 x 10(3)/microL. A white blood cell differential revealed 7% bands, 53% polys, 34% lymphs, 5% atypical lymphocytes, 2% nucleated red cells, and a few young unidentified cells. His chemistry studies were unremarkable. What is the diagnosis?
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PMID:Cases from the Osler Medical Service at Johns Hopkins University. 1521 Mar 89

Two cases of CD56+CD33+ leukemia/lymphoma are reported. The patient in case 1 presented with skin rash, diffuse lymphadenopathy, and hepatosplenomegaly. Blasts with monocytoid and lymphoid features were present in the peripheral blood. The tumor cells expressed HLA-DR, CD4, CD33, CD38, and CD56. Cytogenetic analysis revealed del(2)(p13),del(9)(q22),add(6)(q25),add(12)(p12),-13,-18, and -20. The clinicopathologic features were similar to those of blastic natural killer cell leukemia/lymphoma or type 2 dentritic cell leukemia. The patient in case 2 presented with generalized weakness and skin erythema not responding to antibiotics. Circulating blasts with monocytoid features were seen in the peripheral blood. The tumor cells expressed CD7, CD13, CD33, CD38, and CD56, and cytogenetic analysis revealed -5,add(7)(p22),-8, del(10)(p11.2),-12,der(13; 14)(p10;p10),+14,-16,-18,-19, and del(20)(q13.1). The clinicopathologic features were consistent with a myeloid/ natural killer cell precursor acute leukemia. Both disorders are aggressive hematopoietic malignancies that have similar clinical presentation and morphology but differ in immunophenotype and cytogenetic features.
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PMID:Challenge in diagnosis of CD56+ lymphoproliferative disorders: two cases of CD56+CD33+ lymphoma/leukemia. 1527 May 96


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