A case report of hemophagocytic lymphohistiocytosis (HLH).
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Hemophagocytic lymphohistiocytosis (HLH), is an uncommon, life-threatening hyperinflammatory syndrome caused by severe hypercytokinemia with excessive activation of lymphocytes and macrophages due to a highly stimulated but ineffective immune process. We report a case of Hemophagocytic Lymphohistiocytosis in a 15 year old boy presenting with fever, lymphadenopathy and pancytopenia due to infection caused by Klebsiella Pneumoniae and Acinetobacter.Keywords:
Hemophagocytic Lymphohistiocytosis
Macrophage Activation Syndrome
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Familial hemophagocytic lymphohistiocytosis is a disorder which presents with fever, pancytopenia, liver dysfunction and also an increase in non-malignant histiocytes with prominent hemophagocytosis in various organs. It is usually difficult to distinguish from other hemophagocytic syndrome in the absence of family history. It rarely manifests in adults. Chemotherapy is usually indicated. Here, we report the occurrence of this disorder in two brothers in their twenties.
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Abstract We report a case of chemotherapy‐related acute promyelocytic leukemia (APL) following therapy with VP‐16/etoposide for EBV‐associated hemophagocytic lymphohistiocytosis (HLH). A 17‐month‐old male presented with fever and lymphadenopathy. Bone marrow and liver biopsies showed hemophagocytosis. He responded well to chemotherapy including dexamethasone, VP‐16/etoposide, and cyclosporine. One and a half year later, he developed fever and pancytopenia. Clinical work‐up revealed APL with t(15;17)(q22;q12);PML‐RARα translocation. He underwent chemotherapy for APL and is in remission 8 years after diagnosis. Alternative non‐leukemogenic agents to effectively treat HLH would be desirable. Pediatr Blood Cancer 2011;56:850–852. © 2011 Wiley‐Liss, Inc.
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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, hyperinflammatory syndrome, characterized by the uncontrolled activation of macrophages and T cells, eliciting key symptoms such as persistent fever, hepatosplenomegaly, pancytopenia, hemophagocytosis, hyperferritinemia, and coagulopathy. Viral infections are frequently implicated in the onset of active HLH episodes, both in primary, genetic HLH as in the secondary, acquired form. Infections with herpesviruses such as Epstein-Barr virus and cytomegalovirus are the most common. In autoimmune diseases, a link between viral infections and autoreactive immune responses has been recognized for a considerable time. However, the mechanisms by which viruses contribute to HLH pathogenesis remain to be clarified. In this viewpoint, different factors that may come into play are discussed. Viruses, particularly larger DNA viruses such as herpesviruses, are potent modulators of the immune response. By evading immune recognition, interfering with cytokine balances and inhibiting apoptotic pathways, viruses may increase the host's susceptibility to HLH development. In particular cases, a direct connection between the viral infection and inhibition of NK cell or T cell cytotoxicity was reported, indicating that viruses may create immunological deficiencies reminiscent of primary HLH.
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We present a case of hemophagocytic lymphohistiocytosis (HLH), giving insight in how to establish diagnosis and start appropiate treatment.A 45-year-old male presented at the emergency ward with high fever and pancytopenia. Repeat bone marrow aspirates showed hemophagocytosis. Extensive work-up with exclusion of other infectious and malignant diseases, eventually lead us to the diagnosis of hemophagocytic lymphohistiocytosis.The patient was treated with Etoposide, Cyclosporine A, and systemic steroids based on the HLH-2004 protocol. Unfortunately the patient responded very poorly to the treatment with prolonged pancytopenia, leading to central hemorrhage and eventually death.Diagnosis of hemopagocytic lymphohistiocytosis is difficult and late because of aspecific signs and low incidence. The HLH-criteria can be of use to exclude or confirm diagnosis in unclear cases. Early diagnosis and treatment is crucial given high morbidity and mortality. Therapy depends on type and etiology of the HLH-syndrome. Besides treating the possible triggering factor, Etoposide, steroids, and Cyclosporine A are the mainstays of treatment, sometimes followed by allogenic hematopoietic stem cell transplantation. More research in adults is needed.
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To better define the clinical characteristics and treatment outcomes of autoimmune-associated hemophagocytic syndrome (AAHS) in adults.Adults with AAHS (defined as pathologic evidence of hemophagocytosis without any obvious cause other than an autoimmune disease) were identified through a review of the literature.Among 116 patients identified, underlying diseases included systemic lupus erythematosus (SLE) in 52.3%, adult-onset Still's disease (AOSD) in 26.7%, and dermatomyositis in 6.9%. Fever, lymphadenopathy, hepatomegaly, and splenomegaly were found in 86.8%, 41.0%, 41.8%, and 45.5% of patients, respectively. Cytopenia, liver dysfunction, and hyperferritinemia developed frequently, and coagulopathy was seen in 50.6% of patients. Normal or low C-reactive protein levels were characteristic of patients with underlying SLE. The most commonly used therapy was corticosteroids, which were initially administered in 95.7% of patients, with 57.7% responding. Patients with corticosteroid-refractory disease were usually treated with cyclosporine, intravenous cyclophosphamide (IV CYC), or intravenous immunoglobulin (IVIG), with IV CYC being highly effective. Treatment with biologic agents resulted in favorable effects in the majority of patients. The mortality rate was 12.9%. Male sex (odds ratio [OR] 6.47, 95% confidence interval [95% CI] 2.06-30.39, P < 0.01), dermatomyositis (OR 5.57, 95% CI 1.08-28.65, P < 0.05), and anemia (hemoglobin <8 gm/dl; OR 3.74, 95% CI 1.02-13.8, P < 0.05) were identified as factors associated with mortality.AAHS is potentially fatal. Corticosteroids are a mainstay of initial treatment. For corticosteroid-refractory disease, IV CYC may be beneficial as compared with cyclosporine or IVIG. Treatment that proceeds directly from corticosteroids to biologic agents is promising.
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Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening disorder characterized by an exacerbated but ineffective inflammatory response, which can be classified as primary and secondary HLH. HLH associated with Mycobacterium tuberculosis is uncommon. This case report accounted an immunocompetent patient who was confirmed to be Mycobacterium infection, or rather, highly suspected tuberculosis (TB) associated HLH, with a favorable outcome. Case presentation A 36-year-old man presented with persistent fever, pancytopenia, and hyperferritinemia. A bone marrow smear demonstrated hemophagocytosis, and pathological examination of lung biopsy was positive for acid-fast bacilli, which established the diagnosis of Mycobacterium infection and HLH. Then the patient treated successfully with anti-TB therapy, along with 8 weeks of etoposide. Conclusion This case emphasizes that HLH should be kept in mind when clinicians encounter a patient with severe infection presenting with pancytopenia and hyperferritinemia. Given the high mortality, early diagnosis and appropriate therapy can provide patients with a favorable prognosis.
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Hemophagocytic lymphohistiocytosis is potentially fatal. Prompt diagnosis and initiation of treatment are critical for ensuring the best possible prognosis.We present a case of parvovirus B19 infection related to hemophagocytic lymphohistiocytosis during pregnancy. The patient experienced fever and pancytopenia. A bone marrow biopsy demonstrated hemophagocytosis and a giant proerythroblasts, which is characteristic of a parvovirus B19 infection. Viral serology for parvovirus B19 was positive. Prompt treatment was started because of the high level of certainty of viral-associated hemophagocytic lymphohistiocytosis, and the patient was successfully treated with prednisolone administration. She delivered a healthy newborn without any complications.Hemophagocytic lymphohistiocytosis should be considered when encountering unexplained cytopenia and fever. Prednisolone was an effective treatment.
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Erythema Infectiosum
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TO THE EDITOR:
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive, life-threatening syndrome of excessive immune activation. HLH clinically presents with fever, pancytopenia, splenomegaly, and hemophagocytosis in the bone marrow, lymph nodes, or liver. A proposed mechanism for HLH is a
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A 40-day-old baby girl presented with intermittent fever, lymphadenopathy, massive hepatosplenomegaly, progressive pancytopenia and features of disseminated intravascular coagulopathy. A bone marrow aspiration was performed and showed florid histiocytic proliferation with marked hemophagocytosis. Based on the diagnostic guideline for Hemophagocytic Lymphohistiocytosis proposed by the Familial Hemophagocytic Lymphohistiocytosis Study Group of Histiocyte Society, this patient has fulfilled most of the criteria. We have also found that serum ferritin and lactate dehydrogenase to be very high in this patient. It remains uncertain whether the disorder is reactive or neoplastic.
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