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 authors describe 10 cases of myelofibrosis diagnosed and managed at their center over 16 years. There were 2 and 8 cases, respectively, of primary and secondary myelofibrosis. All patients presented with fever, pallor, hepatosplenomegaly, and/or lymphadenopathy. Hodgkin's lymphoma (n = 4), neuroblastoma (n = 1), thrombasthenic thrombopathy (n = 1), and retroperitoneal-mass (n = 1) were causal in 7 patients, whereas the diagnosis could not be established in a sole case of secondary myelofibrosis. Patients were managed with chemotherapy and appropriate care. However, outcome was poor. The authors emphasize variable clinical-laboratory spectrum of myelofibrosis, highlight management concerns, and demonstrate that prognosis/outcome depends upon appropriate management of the underlying condition.
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PMID:Myelofibrosis in children: experience at a single tertiary care center in India. 2067 Jan 65

Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease caused by defective lymphocyte apoptosis and is characterized by non-malignant lymphoproliferation, hepatosplenomegaly, autoimmune manifestations and increased risk of both Hodgkin's and non-Hodgkin's lymphoma. Most forms of the disease are due to germ line mutations of the FAS gene and manifest during the first years of life with fluctuating lymphadenopathies, hemolysis, immune thrombocytopenia. During the second decade of life disease manifestations improve spontaneously but autoimmune problems still occur and there is an increased risk of lymphoproliferative malignancy. We describe a typical case of ALPS in a now 44 year old man, followed since the age of 2 for disease manifestations that were unclear at the beginning.
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PMID:Autoimmune lymphoproliferative syndrome (ALPS). Case report and family history. 2088 45

Angioimmunoblastic T-cell lymphoma (AITL) is an aggressive non-Hodgkin's nodal peripheral T-cell lymphoma characterized by general lymphadenopathy, night sweats, fever, hepatosplenomegaly, polyclonal hypergammaglobulinemia, and cutaneous involvement. We present a rare case of AITL cutaneous involvement mimicking toxic erythema recurring with AITL relapse and suggesting a precursor of disease progression.
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PMID:Cutaneous involvement in angioimmunoblastic T-cell lymphoma. 2106 26

Hemophagocytic syndrome (HS) is a life-threatening condition of hyperinflammation. Main symptoms are: prolonged fever, cytopenia, hepatosplenomegaly, hemophagocytosis, hyperferritinemia, hypertriglyceridemia and hypofibrinogenemia. Primary genetic form and secondary HS associated with infections, malignancies or autoimmune disorders can be distinguished. Untreated HS in most cases leads to death. We analyzed retrospectively 7 cases of HS in children (3 girls, 4 boys; aged 10 days -14 years) treated in 3 different pediatric centers from 2004 to 2009. In 3 cases HS was associated with infections (EBV, CMV, Bacillus Calmette Guerin - BCG), in 1 child with non-Hodgkin anaplastic large cell lymphoma (ALCL), in 1 patients probably with side effect of antiepileptic drug. In 2 cases cause of HS remained unknown. Fever, hepatomegaly, pan- or bicytopenia and hyperferritinemia were present in all children. In addition, splenomegaly was noted in 6 cases, hemophagocytosis in 6 children, impaired function or decreased number of NK cells in 4 cases, hypofibrino-genemia in 5 and hypotriglyceridemia in 4 patients. Among other symptoms and signs we observed: lymphadenopathy, hepatic failure, oedema, rash, neurological symptoms, increased level of LDH and inflammatory markers. In one child acute pancreatitis occurred. Among others, antibiotics, antiviral and immunosuppressive drugs were used in therapy. HLH-2004 protocol was applied in 4 cases. Patient with ALCL was treated with chemotherapy and allogeneic stem cell transplantation. Four patients are alive, 2 died because of HS, child with ALCL died because of generalized infection in peritrans-plantation period. In case of prolonged fever, splenomegaly and cytopenia diagnosis of HS should be considered. Following tests are recommended: complete blood count, ferritin, triglycerides, fibrinogen, bone marrow aspiration and NK cell assessment. Patients should be also screened for infections and malignancies. Early diagnosis of HS and underlying condition is crucial to start lifesaving therapy.
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PMID:[Hemophagocytic syndrome in children with different underlying conditions]. 2134 76

Chronic lymphocytic leukemia (CLL) is the most common form of leukemia in the Western World. It belongs to the low-grade non-Hodgkin lymphomas and is characterized by clonal reproduction of mature small-cell non-functional B-lymphocytes. CLL affects men somewhat more often than women and the average age at onset is over 50 years. In addition to regional lymph node swelling, typical symptoms include hepatosplenomegaly, leukocytosis and skin disorders such as eczema and pruritus. Manifestations in the oropharynx or hypopharynx are rare but should be kept in mind in the differential diagnosis.
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PMID:[Reduced vocal cord movement and hypopharyngeal tumor]. 2173 80

We present here a case of young male with complaints of fever and swelling in the neck for eight months. History of progressive weakness associated with weight loss was present. Physical examination revealed pallor, multiple enlarged cervical lymph nodes and hepatosplenomegaly. Investigations showed pancytopenia, hyperglobinemia and Leishman-Donovan bodies on bone marrow aspiration. Serological test confirmed diagnosis of visceral leishmaniasis. However, cervical lymph node aspiration and biopsy were suggestive of Mixed cellularity Hodgkin's disease. This made it a very rare case of Leishmaniasis as an opportunistic infection in a patient of pre-chemotherapy Hodgkin's disease. There was marked improvement in haematological profile and regression of hepatosplenomegaly with Amphotericin B treatment followed by favourable response to chemotherapy. The case emphasizes the suspicion for leishmaniasis as a masquerader and as an opportunistic infection in haematological malignancies.
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PMID:Rare association of Visceral leishmaniasis with Hodgkin's disease: A case report. 2201 65

Infectious mononucleosis (IM) is one of the representative, usually benign, acute diseases associated with primary Epstein-Barr virus (EBV) infection. IM is generally self-limiting and is characterized mostly by transient fever, lymphadenopathy and hepatosplenomegaly. However, very rarely primary EBV infection results in severe or fatal conditions such as hemophagocytic lymphohistiocytosis together with fulminant hepatitis designated as severe or fatal IM or EBV-associated hemophagocytic lymphohistiocytosis alone. In addition, chronic EBV-associated diseases include Burkitt's lymphoma, undifferentiated nasopharyngeal carcinoma, Hodgkin lymphoma, T-cell lymphoproliferative disorder (LPD)/lymphoma, natural killer-cell LPD including leukemia or lymphoma, gastric carcinoma, pyothorax-associated lymphoma and senile B-cell LPD as well as chronic active EBV infection and LPD/lymphoma in patients with immunodeficiency. The number of chronic life-threatening diseases linked to the EBV infection is increasingly reported and many of these diseases have a poor prognosis. This review will focus on the historical, pathogenetic, diagnostic, therapeutic and prophylactic issues of EBV-associated life-threatening diseases.
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PMID:Acute or chronic life-threatening diseases associated with Epstein-Barr virus infection. 2210 26

Primary pulmonary Hodgkin's lymphoma (PPHL) is rare. PPHL without peripheral lymphadenopathy or hepatosplenomegaly is exceptionally uncommon. We present a 61-year-old woman believed to have a solitary intrapulmonary fibrous tumour on a CT and a CT-guided biopsy, until surgical excision. Histopathology and immunohistochemistry of the excised mass confirmed PPHL. PPHL is a very rare differential diagnosis of large solitary intrapulmonary masses. A CT-guided biopsy is recommended, as it can be diagnostic, reserving excision for cases where the diagnosis remains in doubt.
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PMID:Giant thoracic mass: an unusual presentation of primary pulmonary Hodgkin's lymphoma. 2404 94

A case of angioimmmunoblastic T-cell lymphoma (AITL) is herein reported. The patient was a 33-year-old female who presented with a tumor arising in the rear of the left lobe of the thyroid. Total removal of the tumor was easily undertaken. Histopathological examinations with Anti-CD3, CD10, CD45RO positive stainings confirmed the diagnosis of AITL. After the determination of stage-categories with IA, 3 courses of chemotherapy with CHOP were administered followed by X-ray irradiation of the cervix with 40 Gy. Thereafter close follow-up has not revealed any recurrence. Serum levels of soluble IL-2 receptor (sIL-2R) gradually decreased according to the round of treatments. AITL is a rare clinical entity included in 1.2-2.5% of non-Hodgkin's lymphomas, which has not at all been reported in the issues concerning otorhinolaryngology. Clinical symptoms and findings include general lymphoadenopathy, hepatosplenomegaly, skin rash, anemia and hypergannmagloburinemia. Diagnosis is determined by histopathological, immunohistochemical and clonality examination. Chemotherapy regimens using multi-agents combined with anthracycline i.e. CHOP have been playing the fundamental role in the therapeutic paradigm, but because of the poorer survival of 20-50% at the 5-year point, AITL is classified as a high-grade malignancy.
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PMID:[A case of angioimmunoblastic T-cell lymphoma arising in the cervical region]. 2431 62

Hemophagocytic lymphohistiocytosis (HLH) is a severe life-threatening disorder, responsible for extensive phagocytosis of hematopoietic cells and causing a multisystem organ failure. If lymphomas are common causes of HLH, the association with Hodgkin's lymphoma is rarely described in children. We report a case of a 9-year-old boy presenting with HLH as an initial manifestation of Hodgkin's lymphoma. He has been suffering from persistent high fever, asthenia, weight loss, and hepatosplenomegaly with no lymphadenopathy. The diagnosis of HLH secondary to infectious disease was initially worn. The patient received high-dose intravenous immunoglobulin with broad-spectrum antibiotics. However, his state got worse with the onset of dry cough and pleural effusion. Histopathologic examination of pleural fluid showed the presence of Reed-Sternberg cells. The outcome was favorable after treatment by corticosteroid and chemotherapy. Hodgkin's lymphoma revealed by HLH is a source of delayed diagnosis and should be borne in mind in children.
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PMID:Hodgkin's Lymphoma Revealed by Hemophagocytic Lymphohistiocytosis in a Child. 2532 42


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