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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemophagocytic lymphohistiocytosis (HLH) is a heterogeneous disorder characterized by excessive activation and proliferation of nonmalignant histiocytes, which are commonly found in bone marrow, lymph nodes, spleen and liver in affected patients. Here, we report the presence of glomerular macrophages, including one showing erythrophagocytosis, on renal biopsy in a 25-year-old patient with clinical presentation and laboratory changes consistent with HLH. The clinical course was marked by persistent fever for 2 months, pleural and pericardial effusion, splenomegaly, lymphadenopathy, pancytopenia, cardiac arrhythmias, multiple organ dysfunction, and proteinuria, with demise after a 2-month hospitalization. Positive assay for Epstein-Barr virus (EBV), marked hyperferritinemia, hypofibrinogenemia, hypertriglyceridemia, elevated anti-nuclear antibody, proteinuria, and decreased circulating NK cells by flow cytometry were compatible with the diagnosis of HLH. We suggest that the glomerular hemophagocytic macrophages, which have not heretofore been described in the kidney of a patient with HLH, may have contributed to renal dysfunction manifest as proteinuria.
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PMID:Glomerular hemophagocytic macrophages in a patient with proteinuria and clinical and laboratory features of hemophagocytic lymphohistiocytosis (HLH). 2203 68

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder of immnoregulation caused by various inherited and acquired factors. Uncontrolled activation of T lymphocytes and macrophages, together with an impaired cytotoxic function of NK cells, results in the massive cytokine release and hyperinflammatory phenotype. HLH is characterized by fever, splenomegaly and peripheral blood cytopenia. Cytohistological examination shows accumulation of lymphocytes and macrophages, sometimes with hemophagocytic activity, in different tissues (e.g., bone marrow). Laboratory findings include hyperferritinemia (often 'sky-high' > 10.000 microg/l), hypertriglyceridemia, hypofibrinogenemia, and high levels of the alpha chain of the soluble interleukin-2 receptor (sIL-2R also called sCD25). In 2004 The Histiocyte Society updated current diagnostic guidelines for HLH. Therapy of HLH is based on suppression of the hyperinflammatory status by destruction of activated CD8+ T lymphocytes and macrophages, and treatment of any existing HLH triggers. However, treatment of HLH is associated with high morbidity and mortality. Therefore consideration should be given to referring HLH patients to centers with experience in the treatment and care of those with HLH. Due to aggressive course, awareness of HLH signs and symptoms is crucial among physicians for the early diagnosis and immediate introduction of adequate HLH treatment.
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PMID:[Hemophagocytic lymphohistiocytosis--a contemporary medical problem]. 2240 Jan 83

Hemophagocytic syndrome (HPS) may be provoked by infections, malignancies and autoimmune diseases. We report on a 56-year-old woman with long-lasting systemic lupus erythematosus (SLE) who presented with malar rash, inflammatory livedo reticularis, fever, weight loss, pancytopenia and mild splenomegaly with cervical lymphadenopathy. She had criteria for SLE flare-up (malar rash, high antinuclear antibody titer, complement consumption, pathological urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid therapy, pancytopenia and fever worsened. Important elevations of triglycerides and ferritin were also found. Bone marrow aspirate demonstrated hemophagocytosis, which confirmed the coexistence of HPS and SLE. The treatment with glucocorticoids, immunoglobulins, cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful. After one month, the patient developed Pneumocystis jirovecii pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days before death, showed high grade diffuse large B-cell (CD20+, Ki-67+) non-Hodgkin's lymphoma (DLBCL). We are the first to report the association of both SLE and non-Hodgkin's lymphoma complicated by HPS. We showed that, based on clinical and laboratory data, it was difficult to distinguish the early phase of HPS from SLE flare-up and new-onset DLBCL. Therapy of such a complex case of HPS has not been standardized, and opportunistic infections remain a difficult issue.
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PMID:Systemic lupus erythematosus progressing to non-Hodgkin's lymphoma complicated by fatal hemophagocytic syndrome: case report. 2250 70

A 5-month old infant presented with a short history of fever of unknown origin. He initially appeared well although there was a resting tachycardia during periods of normal temperature, and pancytopenia. He remained febrile in spite of antibiotic therapy and by 48 h he had developed marked hepatosplenomegaly and coagulopathy. At 72 h a bone marrow aspirate and trephine biopsy showed haemophagocytosis. By this time the infant was encephalopathic and haemodynamically unstable with multi-organ dysfunction. Treatment with high-dose methylprednisolone and cyclosporin was commenced with an initial good response. Unfortunately the patient ultimately died of infective complications of treatment and reactivation of the underlying disease process. Haemophagocytic lymphohistiocytosis (HLH) is a rare disorder of the mononuclear phagocyte system characterised by proliferation of morphologically benign histiocytes resulting in hypercytokinaemia and uncontrolled activation of immune cells. The diagnosis of familial HLH should be considered in any infant with fever, splenomegaly and cytopenia of at least two cell lines. HLH may be cured by immunosuppressive therapy and eventual stem cell transplantation but rapid disease progression to multi-organ failure results in a high mortality.
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PMID:An unusual cause of fever. 2300 94

We report a case of a 68-year-old female patient who developed hemophagocytic lymphohistiocytosis (HLH) secondary to peripheral T-cell lymphoma (PTCL) not otherwise specified (NOS) that developed in the setting of treatment-resistant B-cell small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL). The patient's B-cell lymphoma had a good initial response to chemotherapy for 4 years, after which it became less responsive and was thought to have undergone transition to a higher-grade lymphoma. Different regimens of chemoradiotherapy were then tried with modest response until the patient presented 3 years later with signs and symptoms of HLH. The patient died 1 month later, and an autopsy was performed. Significant para-aortic lymphadenopathy and splenomegaly were found. Microscopic, immunohistochemical and molecular evaluations confirmed the presence of composite B-cell and T-cell lymphoma in the para-aortic enlarged lymph nodes. Bone marrow examination showed hemophagocytosis, and the liver demonstrated infiltration by activated macrophages with hepatocellular necrosis. This report highlights the importance of searching for a possible underlying T-cell lymphoma in light of HLH. Different theories have been proposed to explain the rare occurrence of concurrent B- and T-cell lymphomas, but the development of HLH in this patient highlights the importance of immune dysregulation as a proposed mechanism to explain some cases of composite lymphomas. A review of the literature and discussion of the relative merits of these hypotheses are presented in the context of this case.
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PMID:Composite peripheral T-cell lymphoma not otherwise specified, and B-cell small lymphocytic lymphoma presenting with hemophagocytic lymphohistiocytosis. 2312 36

Hemophagocytic lymphohistiocytosis (HLH) is a severe inflammatory disorder characterized by activation and proliferation of lymphocytes and histiocytes with cytokine release and uncontrolled hemophagocytosis, especially late in the course of the disease. Clinical features include relapsing fevers, hepatosplenomegaly, cytopenias, lymphadenopathy, and coagulopathy. The diagnosis can be challenging, as the early signs and symptoms are nonspecific and no specific laboratory tests exist. This syndrome is frequently not recognized and has a significant mortality rate. Typical scenarios in which HLH should be considered include mononucleosis (fever, hepatosplenomegaly, and lymphadenopathy) in an infant or young child, aseptic meningitis associated with cytopenias, or a viral syndrome-like illness with cytopenias and lymphadenopathy or splenomegaly, for example. Our approach includes measuring a ferritin level as a screening tool early in the course of such an illness. Two cases of HLH are reviewed, illustrating the frequent complexity of these cases and potential pitfalls to making a prompt diagnosis.
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PMID:Hemophagocytic lymphohistiocytosis--a diagnostic dilemma: two cases and review. 2317 2

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome (HPS), is a rare, life-threatening, hematologic disorder manifested by clinical findings of extreme inflammation and unregulated immune activation. In both its congenital (primary) and adult (secondary) forms, it is most often characterized by fevers, hepatomegaly or splenomegaly, and bi- or trilineage cytopenias. In addition, elevated liver enzymes, hyperferritinemia, hypertriglyceridemia, and hypofibrinogenemia are commonly seen in HLH patients. A high index of suspicion is necessary for early diagnosis. Furthermore, a thorough diagnostic evaluation is necessary, and prompt treatment of the underlying causes is key in order to prevent irreversible tissue damage. Here we discuss the clinical signs, diagnosis, and treatments associated with this rare and potentially lethal disorder as manifested in adults.
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PMID:Secondary hemophagocytic lymphohistiocytosis in adults: an update on diagnosis and therapy. 2327 Dec 59

Haemophagocytic syndrome or haemophagocytic lymphohistiocytosis is a disorder of histiocytes that has sepsis-like features, combined with haemophagocytosis, cytopenias, hyperferritinaemia, hypercytokinaemia and splenomegaly. Diagnostic, therapeutic and prognostic guidelines are available for childhood (familial) haemophagocytic syndrome. The disorder is diagnosed less frequently among adults than children. We report a case of Epstein-Barr virus-induced haemophagocytic syndrome in a 23-year-old man, who responded to treatment with steroids and chemotherapy.
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PMID:Epstein-Barr virus-induced haemophagocytic syndrome. 2344 26

Hemophagocytic lymphohistiocytosis (HLH) is rarely described in association with chronic lymphocytic leukemia (CLL), mostly triggered by disease progression or concurrent infection. A 68-year-old male received 4 cycles of fludarabine, cyclophosphamide, and rituximab (FCR) for CLL and achieved a complete response. Twenty-four days after the last chemotherapy, he presented with febrile neutropaenia and was diagnosed with HLH. The diagnosis was based upon persistent fever, pancytopenia, hyperferritinemia, splenomegaly, and hemophagocytosis on bone marrow aspirate. He began treatment with dexamethasone, cyclosporine, and etoposide. Fever resolved and hyperferritinemia improved but pancytopenia persisted. He died 13 days later from septic shock with positive blood cultures. A limited postmortem examination was performed and biopsies were taken from bone marrow, liver, and spleen. Biopsies demonstrated abundant hemophagocytosis by the activated macrophage as stained by CD68. There was no evidence of residual CLL as demonstrated by the lack of lymphocytes which was confirmed by the negative staining of CD79a. Chemotherapy appears to be responsible for the development of HLH in this patient. This is the second reported case of HLH developing after a rituximab-containing chemotherapy.
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PMID:A case of hemophagocytic lymphohistiocytosis in a patient with chronic lymphocytic leukemia after treatment with fludarabine, cyclophosphamide, and rituximab chemotherapy, with autopsy findings. 2353 46

Hemophagocytic syndrome (HPS) is an uncommon hematological disorder that manifests as fever, splenomegaly, and jaundice, with hemophagocytosis in the bone marrow and other tissues pathologically. Secondary HPS is associated with malignancy and infection, especially viral infection. The prevalence of cytomegalovirus (CMV) infection in ulcerative colitis (UC) patients is approximately 16%. Nevertheless, HPS in UC superinfected by CMV is very rare. A 52-year-old female visited the hospital complaining of abdominal pain and hematochezia for 6 days. She was diagnosed with UC 3 years earlier and had been treated with sulfasalazine, but had stopped her medication 4 months earlier. On admission, her spleen was enlarged. The peripheral blood count revealed pancytopenia and bone marrow aspiration smears showed hemophagocytosis. Viral studies revealed CMV infection. She was treated successfully with ganciclovir. We report this case with a review of the related literature.
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PMID:A case of hemophagocytic syndrome in a patient with fulminant ulcerative colitis superinfected by cytomegalovirus. 2368 30


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