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 26-year-old female patient developed high fever, hepatosplenomegaly and subcutaneous nodules. Atypical lymphocytes were present in the peripheral blood. Skin biopsy revealed lobular panniculitis. Bone marrow examination showed prominent phagocytosis by benign histiocytes. The diagnosis of cytophagic histiocytic panniculitis was made, and the disease has been well controlled with oral prednisolone. This is the first report of cytophagic histiocytic panniculitis with atypical lymphocytes in the peripheral blood, which are frequently seen in virus-associated hemophagocytic syndrome. This observation suggests that underlying viral infection may be one factor in the development of cytophagic histiocytic panniculitis.
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PMID:Cytophagic histiocytic panniculitis with atypical lymphocytes in peripheral blood. 129 32

A 21-year-old woman had suffered from repeated vasculitis and panniculitis with fever and chills after following mosquito bites since age 7. These manifestations were confirmed with a direct mosquito bite test during her admission to another hospital at age 17. The patient presented to our hospital with progressive dyspnea, productive cough and intermittent fever for one week. In addition to the bilateral infiltrative lesions on chest roentgenography, hepatosplenomegaly with an abnormal liver function test, pancytopenia, and elevated IgE were also detected. Blood and sputum cultures grew no microorganisms. Epstein-Barr virus-IgM, Cytomegalovirus-IgM and Mycoplasma pneumonia antibodies were all negative. Bone marrow aspiration and biopsy revealed histiocytosis with hemophagocytosis. No atypical histiocyte was found. The patient was put on dexamethasone treatment with improvement. Unfortunately, symptoms relapsed two weeks later. A repeated bone marrow aspiration and biopsy revealed a picture similar to the previous one. Despite antibiotic administration and ventilator support, a rapidly deteriorated course terminated in the patient's death by respiratory failure. Mosquito bite allergies were reported to be associated with malignant histiocytosis in Japan. However, atypical histiocytes were not found in our case upon repeated bone marrow aspirations, biopsies and skin biopsies. Mature histiocytes with hemophagocytosis were prominent instead. Reactive histiocytosis was thus favored rather than malignant histiocytosis. Although opportunistic infection cannot be excluded, we propose that a mosquito bite allergy with consequent histiocytic activation by antigen, immune complex or IgE is the possible pathogenetic mechanism for hemophagocytic histiocytosis in this patient.
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PMID:[Mosquito bite allergies terminating as hemophagocytic histiocytosis: report of a case]. 257 73

Two patients with benign cytophagic histiocytic panniculitis are presented and compared with the one benign and ten fatal cases previously reported. Benign-appearing histiocytes which engulf blood cells, nuclear debris, and platelets are the most characteristic feature of the disease. This cytophagia tends to be massive in the subcutaneous tissue and involves extracutaneous organs only in the fatal cases. The course is chronic in the benign form. Patients with the fatal form tend to have fever, hepatosplenomegaly, serosal effusions, ecchymoses, peripheral adenopathy, and mucosal ulcers. Anemia, leukopenia, elevated liver enzyme levels, and coagulopathy are present in almost all and hypocalcemia in many patients with a fatal course. Cytophagic histiocytic panniculitis may represent a form of regional histiocytosis primarily involving subcutaneous tissue. It is midway in the spectrum of the cytophagic histiocytoses.
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PMID:Cytophagic histiocytic panniculitis is not always fatal. 276 94

Five patients had a histiocytic, cytophagic panniculitis and a febrile, progressive course, which included hepatosplenomegaly, pancytopenia, liver function abnormalities, and a hemorrhagic death. The terminal hemorrhage was characterized by features of intravascular coagulation and liver failure. The histiocytosis could be found at times also in bone marrow, lymph nodes, liver and spleen, and serosal tissues, as well as in skin and subcutaneous tissue. Adequate biopsy diagnosis will prevent such cases from being labeled as Weber-Christian disease with bleeding, and offer an opportunity for appropriate treatment of this new syndrome of histiocytic, cytophagic panniculitis.
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PMID:Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis. 743 42

Two cases of what was originally called "Weber-Christian disease" were found to be a chronic, recurrent form of histiocytic panniculitis characterized by histiocytic engulfment of red and white blood cells--"cytophagocytosis." The disease was manifested by recurrent fever, subcutaneous nodules, pancytopenia, and mucosal ulcerations. Hepatosplenomegaly and enlarged lymph nodes were observed. Focal invasion of the reticuloendothelial system by acidophilic or cytophagic histiocytes could be identified. Progressive changes in liver function and hemorrhagic diathesis occurred in both patients.
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PMID:[Panniculitis with cellular phagocytosis. Chronic form of histiocytic panniculitis with fever, pancytopenia, polyserositis and lethal hemorrhagic diathesis]. 745 Nov 40

A 20-year-old Japanese man developed generalized, subcutaneous, painless nodules, fever, abnormal liver function, serosal effusions, hepatosplenomegaly, lymphadenopathy and anemia. Skin biopsies revealed lobular panniculitis with a morphologically benign histiocytic infiltration and prominent phagocytosis. Atypical T lymphocytes were also present in the skin and liver. The diagnosis given was aggressive cytophagic histiocytic panniculitis (CHP) or aggressive subcutaneous panniculitic T cell lymphoma (SPTCL). He received cyclophosphamide, doxorubicin, and vincristine on day 1, prednisolone on days 1-5, and etoposide on days 1, 3 and 5 (CHOP-E), with the support of granulocyte colony-stimulating factor. This regimen was repeated every 2 weeks and complete clinical remission (CCR) was attained after three cycles of CHOP-E. As the clinical course of aggressive CHP is recurrent and often fatal, he was given high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (APBSCT), after five cycles of CHOP-E. He has remained in CCR for 12 months after APBSCT. High-dose chemotherapy followed by APBSCT is considered to be one of the most beneficial therapies for patients with aggressive CHP and aggressive phase SPTCL.
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PMID:Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis. 924 23

We report a 35-year-old man, who had been diagnosed with Weber-Christian disease, presented with acute onset of high fever, malaise, jaundice and hepatosplenomegaly with subcutaneous nodules. Laboratory tests showed elevated serum ferritin and liver enzymes, especially lactate dehydrogenase (LDH), with pancytopenia and coagulation abnormalities. Peripheral blood and bone marrow examinations showed erythro-, leuko- and thrombo-phagocytic histiocytes and macrophages. The patient developed the same clinical features seven years ago. Based on diagnosis of cytophagic histiocytic panniculitis, the patient was treated with steroid pulse therapy and oral cyclosporin A. The combination therapy caused a marked improvement in the clinical condition.
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PMID:A case of cytophagic histiocytic panniculitis: successful treatment of recurrent attacks with steroid pulse therapy and oral cyclosporin A. 925 59

We present two fatal cases of cytophagic panniculitis (CP) and haemophagocytic syndrome (HPS). In the first of these, there was an underlying T-cell lymphoma but in the second no associated disease was found. HPS is a frequently fatal disorder of immune regulation, characterized by fever, histiocytic haemophagocytosis, hepatosplenomegaly, pancytopenia, hypertriglyceridaemia and coagulopathy; CP is a less common manifestation. A number of benign and malignant conditions may present with HPS, the clinical findings and investigations aiding in determining an underlying disorder. Therapy is both supportive and directed at any associated illness, but often very difficult as diagnosis is delayed.
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PMID:Fatal cytophagic panniculitis and haemophagocytic syndrome. 969 4

Haemophagocytic syndrome is a rare disorder of immune regulation. Clinical features are fever, haemophagocytosis, hepatosplenomegaly, pancytopenia, lymph nodes enlargement, hypertriglyceridaemia and coagulopathy. Systemic findings may also include pulmonary infiltrates, renal failure and an inappropriate antidiuretic state. It has been described associated to many diseases, like lymphomas, most of them T-cell lymphomas, infections and systemic diseases, among other entities. Haemopoietic cells are actively ingested by monocytes and macrophages in lymph nodes, bone marrow, liver and spleen. This is a diagnostic criteria for haemophagocytic syndrome. The treatment of haemophagocytic syndrome is difficult and requires intensive supportive therapy, treatment of coagulopathy disorders, high-dose corticosteroids and immunosuppressive treatment, although in most cases there is no response to treatment and the evolution is fatal. The T-cell lymphomas that involve subcutaneous tissue and simulate panniculitis are classified recently as primary cutaneous T-cell lymphomas, and their presentation as inflammatory nodules of the legs are uncommon.
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PMID:[Haemophagocytic syndrome and cutaneous T-cell lymphoma]. 1504 94

Generalized lipodystrophy (GLD) is characterized clinically by an almost complete lack of fat in adipose tissue. Traditionally, GLD can be divided into congenital and acquired types according to the clinical course and underlying etiologies. Here, we describe a boy with a probable diagnosis of acquired GLD. He showed recurrent panniculitis since early infancy, and short stature with delayed skeletal age (3 years less) and low levels of insulin-like growth factor-1 (IGF-1) and IGF binding protein-3. Growth hormone (GH) deficiency was confirmed by GH provocative tests (peak GH below 5 microg/L). In addition to slow progressive loss of subcutaneous fat tissue, he also suffered from hepatosplenomegaly, recurrent febrile episodes in association with painful nodular skin lesions and abdominal pain. The diagnosis of lipodystrophic panniculitis was confirmed after skin biopsy. The febrile episodes and skin lesions responded to oral corticosteroid with the progression of time. Acquired GLD with GH deficiency (e.g., recurrent panniculitis) may occur as a result of chronic inflammation over the pituitary stalk and pituitary gland. The use of steroid and GH replacement may alleviate this disorder.
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PMID:Recurrent panniculitis associated with generalized lipodystrophy and growth hormone deficiency. 1609 31


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