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)

The occurrence of T system immunodeficiency in an infant together with excessive production of IgM and, to a lesser degree, of IgG and IgA, is an unusual combination. A case is reported in which an unremitting lung infection with lymphadenopathy and hepatosplenomegaly developed in a previously healthy two-month-old infant. Leukocytosis with lymphocytosis, monocytosis and eosinophilia was rapidly followed by leukopenia and lymphocytepenia after a blood transfusion for anemia. There was a transient clinical remission, but on relapse 10 days later, quantitative and functional T cell deficiency was found together with increased IgG and IgA and with IgM values reaching 50 times greater than normal. Thymic humoral factor was successful in vitro in increasing the number of identifiable T cells (E rosetts) as well as T cell function (leukocyte migration inhibition factor production). However, the infant died suddenly, and at autopsy evidence of a generalized inflammatory reaction compatible with a viral infection was found. The thymus was small, hypoplastic and hypocellular. It is speculated that the T system deficiency may have been acquired following Epstein-Barr virus infection, and that T cell regulatory activity of immunoglobulin production was defective.
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PMID:Immune deficiency of T system with possible T cell regulatory activity defect. 19 69

A 47-year-old white male developed massive hepatosplenomegaly, a pleural effusion, leucocytosis, and a left parasternal mass following a relatively symptom-free persistent hypereosinophilia for about 5 years. Bone marrow aspiration and biopsy and peripheral blood differential showed eosinophilia and a shift to the left with immature cells. A high serum B12 vitamin level and low LAP activity were found. Biopsy of the soft tissue mass revealed a granulocytic sarcoma (chloroma) with a hyperdiploid karyotype (49,XY, + 10, + 15, + 19,3q-), whereas the bone marrow cells had a normal male karyotype. The patient responded temporarily to chemotherapy but eventually developed CNS leukemia and went on to terminate in a frank blastic phase. This case illustrates hypereosinophilia and a myeloproliferative syndrome characterized by a somewhat indolent chronic course evolving into "eosinophilic leukemia" and granulocytic sarcoma, CNS involvement by leukemic cells and, finally, blastic transformation. It is possible that this case represents a variant of Ph1-negative CML to which the term "chronic eosinophilic leukemia" could be justifiably applied.
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PMID:Chromosomes and causation of human cancer and leukemia. XXXIV. A case of "hypereosinophilic syndrome" with unusual cytogenetic findings in a chloroma, terminating in blastic transformation and CNS leukemia. 29 66

Immunoblastic lymphadenopathy was first described in the german literature in 1975 by Lennert as "Lymphogranulomatosis X." The disease is characterized by generalized lymphadenopathy, hepatosplenomegaly, dysproteinemia, fever and hyperergic reactions such as pruritus, skin rush and eosinophilia. The first ORL occurrence of the disease in Waldeyer's ring and cervical lymph nodes is reported. The histologic picture is characterized by a vascular proliferation with immunoblasts, plasma cells and interstitial amorphous acidophilic material but without Sternberg cells. The origin of the disease from primary malignant neoplasia or hyperergic reactions has not yet been defined. Although prognosis is uncertain and therapy limited, the best management involves small doses of corticoids, supplemented by antibiotics.
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PMID:[Head and neck manifestations of lymphogranulomatosis X (author's transl)]. 70 Nov 5

Five patients were seen at the Mayo Clinic over an 8-year period with the following complex of clinical and morphologic features; striking eosinophilia, cardiomyopathy, hepatosplenomegaly, and either a rapidly fatal or a prolonged, debilitating illness. In recent years, controversy has raged over the precise designation of this syndrome, with proposals ranging from eosinophilic leukemia to hypereosinophilic syndromes. To focus on the major target organ of the disease, we have favored the term endomyocardiopathy with eosinophilia. Experience with these five patients showed that (1) eosinophilia can persist for many years before symptoms appear; (2) progressive restrictive cardiac disease was the major cause of death and debility; (3) osmiophilic cytoplasmic inclusions are present in eosinophils of these patients and also in cells from other patients with marked eosinophilia; and (4) echocardiography may prove to be a useful noninvasive tool to diagnose and follow the progress of cardiac involvement. Although none of these patients was thought to have leukemia, intensive therapy with steroids or cytotoxic agents, or both, is considered necessary to control the progression of the disease.
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PMID:Endomyocardiopathy with eosinophilia. 99 51

We describe a 13-year-old girl with angioimmunoblastic lymphadenopathy. The patient's main symptom was a generalized pruritic maculopapular rash located mainly on the upper and lower limbs. In addition to the skin lesions, physical examination revealed enlarged cervical, axillary and inguinal lymph nodes. There were also hepatosplenomegaly and oedema of both hands. Blood examination showed elevated ESR, haemolytic anaemia, polyclonal hypergammaglobulinaemia and eosinophilia. Virus serology including HIV I and II and HTLV I was negative. Histopathological examination of a lesional skin biopsy showed superficial and deep dermal infiltrate extending into the subcutaneous tissue. The infiltrate consisted of lymphocytes, some with atypical nuclei, histiocytoid cells, and few eosinophils. There was also proliferation of dermal blood vessels. Examination of an enlarged cervical lymph node disclosed typical histopathological features of angioimmunoblastic lymphadenopathy and confirmed the diagnosis.
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PMID:[Angioimmunoblastic lymphadenopathy with cutaneous manifestations in a 13-year-old girl]. 128 12

The case of a two-year-old girl with generalized histiocytosis, probably induced by phenobarbital, is reported. Symptoms, including intermittent fever, systemic lymphadenopathy, maculopapular skin eruption and hepatosplenomegaly, suggested Langerhans cell histiocytosis. Laboratory examinations revealed leukocytosis with lymphocytosis and eosinophilia and a high LDH serum level, while GOT and GPT were within normal ranges. Cytological studies of lymph node and pleural effusion specimens revealed proliferation and infiltration of Langerhans cell histiocytes with eosinophilia. No histiocyte proliferation was observed in the bone marrow or skin. The clinical manifestations shown by the patient were, however, transient, and improved spontaneously after the discontinuation of phenobarbital. The case was considered to be one of phenobarbital hypersensitivity syndrome based on clinical course and laboratory findings. The mechanism and differential diagnosis of the syndrome are discussed.
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PMID:A case of hypersensitivity syndrome resembling Langerhans cell histiocytosis during phenobarbital prophylaxis for convulsion. 129 59

The primary complex like Ghon was observed in a child's clinical roentgenographic study. C.S., white, male, 6 years old, was born in Curitiba (PR), Brazil and living in Guaratingueta (SP), Brazil, developed "common cold", bimodal diary fever, chills, shake and sweats. Dyspnea, cough with general lymphadenopathy. Foot and right shoulder arthralgias. Six months ago visited a cave, equitation practice, dog and cat contacts and no transfusion, frontal sweats, fever (38.4 degrees C). T.A. was 8/6, tachycardia in generalized lymphadenopathy. Cardiopulmonary system was normal, mesogastric tumoral mass, hepatosplenomegaly and no ascites. Bone marrow with eosinophilia; nodule demonstrated presence of P. brasiliensis, hypoalbuminemia; hyperglobulinemia; anemia; leukocytosis with eosinophilia. Immunodiffusion with exoantigen 43 kd of P. brasiliensis was 1/32. Primary complex like Ghon was observed in interstitial pneumonia followed by mediastinal and mesogastric mass (35 to 40 days). Clavicular osteolytic lesions (45 to 60 days) appeared during paracoccidioidomycosis therapy. Recovery was observed 2 months after treatment of acute infantile paracoccidioidomycosis.
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PMID:[Pulmonary lymph node in acute juvenile paracoccidioidomycosis (a case report)]. 130 53

A 36-year-old male was admitted to the Ehime University Hospital with anemia, eosinophilia and hepatosplenomegaly. Peripheral blood examination demonstrated severe anemia (Hb 7.1g/dl), thrombocytopenia (Plt 6.8 x 10(4)/microliters) and increase of peripheral leukocyte counts (53,000/microliters) with 32.0% of eosinophils which had lobulated nuclei, abnormal distribution of eosinophilic granules and a few vacuoles. The level of serum IgE was low (< 5IU/ml), while that of serum vitamin B12 was elevated. A diagnosis of eosinophilic leukemia was made. He was noted to have spontaneous fluctuations in his eosinophil and total leukocyte counts. To analyze the mechanism of cyclic eosinophilic leukocytosis, we examined eosinophil colony stimulating activity of the serum and plasma of the patient. These examination showed that eosinophil colony-stimulating activity was not found in his serum and plasma, and cyclic eosinophilic leukocytosis was due to the hemopoietic stem cell disorder.
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PMID:[Eosinophilic leukemia with cyclic eosinophilic leukocytosis]. 147 3

We report the case of a 14-year-old Japanese boy with peripheral T-cell malignant lymphoma, showing progression from immunoblastic lymphadenopathy (IBL) to overt malignant lymphoma. He suffered recurrent fever, generalized lymphadenopathy, hepatosplenomegaly and maculopapular exanthema. Leukocytosis with eosinophilia and polyclonal hypergammaglobulinemia were observed during the aggressive course of the disease. In the early phase, human immunoglobulin and steroids improved the symptoms but did not induce complete remission, and the patient died one year after the onset of the illness. Four biopsies of lymph nodes revealed progression from IBL to CD4 positive T-cell lymphoma through IBL-like T-cell lymphoma. Though IBL-like T-cell lymphoma is defined as IBL with neoplastic features and overt T-cell malignant lymphoma progressed from IBL-like T-cell lymphoma is excluded from the definition, it may be preferable that such malignant lymphoma as our case should also be included in IBL-like T-cell lymphoma.
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PMID:Immunoblastic lymphadenopathy (IBL)-like T-cell lymphoma in a child. 178 38

A 4-month-old male infant had a 2-month history of an exfoliative erythroderma and alopecia. Recurrent mucosal infections, diffuse lymphadenopathy, hepatosplenomegaly, lymphocytosis and eosinophilia, anemia, and failure to thrive later developed. Investigation revealed a combined immunodeficiency with T cells of an unusual phenotype in his peripheral blood, skin, and lymph nodes. Our patient's clinical manifestations most closely resemble Omenn's syndrome, a rare form of autosomal recessive combined immunodeficiency.
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PMID:Omenn's syndrome and related combined immunodeficiency syndromes: diagnostic considerations in infants with persistent erythroderma and failure to thrive. 183 95


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