Ehrlichia-Induced Hemophagocytic Lymphohistiocytosis in Two Children
ABSTRACT Two children presented with a history of fever and rash. Lab values revealed pancytopenia, elevated ferritin, coagulopathy, and elevated triglycerides. Both children quickly developed respiratory distress and hypotension requiring admission to the ICU. Bone marrow biopsies revealed hemophagocytosis. Studies for Ehrlichia returned positive. The patients were started on doxycycline and treated for hemophagocytic lymphohistiocytosis (HLH). Each made a full recovery. In both patients, testing for MUNC and perforin genes were found to have no mutation. These two cases demonstrate the importance of considering Ehrlichiosis as a possible trigger of HLH.
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ABSTRACT: Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening conditions featuring ineffective immunity characterized by an uncontrolled hyperinflammatory response. HLH is often triggered by infection. Familial forms result from genetic defects in natural killer cells and cytotoxic T-cells, typically affecting perforin and intracellular vesicles. HLH is likely under-recognized, which contributes to its high morbidity and mortality. Early recognition is crucial for any reasonable attempt at curative therapy to be made. Current treatment regimens include immunosuppression, immune modulation, chemotherapy, and biological response modification, followed by hematopoietic stem-cell transplant (bone marrow transplant). A number of recent studies have contributed to the understanding of HLH pathophysiology, leading to alternate treatment options; however, much work remains to raise awareness and improve the high morbidity and mortality of these complex conditions.Hematology Research and Reviews 01/2014; 5:69-86. DOI:10.2147/JBM.S46255
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ABSTRACT: BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome that is often fatal despite treatment. It is caused by a dysregulation in natural killer T-cell function, resulting in activation and proliferation of histiocytes with uncontrolled hemophagocytosis and cytokines overproduction. The syndrome is characterized by fever, hepatosplenomegaly, cytopenias, liver dysfunction, and hyperferritinemia. HLH can be either primary, with a genetic aetiology, or secondary, associated with malignancies, autoimmune diseases, or infections. AIM: To focus on secondary HLH complicating zoonotic diseases. MATERIALS AND METHODS: PubMed search of human cases of HLH occurring during zoonotic diseases was performed combining the terms (haemophagocytic or haemophagocytosis or hemophagocytosis or hemophagocytic or erythrophagocytosis or macrophage activation syndrome) with each one of the etiological agents of zoonoses. RESULTS: Among bacterial diseases, most papers reported cases occurring during brucellosis, rickettsial diseases and Q fever. Regarding viral diseases, most of the cases were reported in patients with avian influenza A subtype H5N1. Among the protozoan zoonoses, most of the cases were reported in patients with visceral leishmaniasis. Regarding zoonotic fungi, most of the cases were reported in AIDS patient with histoplasmosis. No cases of secondary HLH were reported in patient with zoonotic helminthes. CONCLUSIONS: Zoonotic diseases are an important cause of HLH. Secondary HLH can delay the correct diagnosis of the zoonotic disease, and can contribute to an adverse outcome.European review for medical and pharmacological sciences 10/2012; 16(10):1324-1337. · 0.99 Impact Factor
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ABSTRACT: Objectives To present a case of human monocytic ehrlichiosis (HME) that was complicated by macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis (sHLH). Methods Data was collected from patient's electronic medical records at the University of Alabama at Birmingham. The patient is a part of a larger cohort of patients with all-cause MAS treated at our center. Case A 63 year old renal transplant recipient male on maintenance immunosuppressive therapy presented with high grade fever, leukopenia, thrombocytopenia and elevated transaminases and initially met clinical criteria for severe sepsis. On further investigation, clinical and laboratory criteria for MAS were met. He was treated with a combination of doxycycline for HME and a novel combination of anakinra (interleukin-1 receptor antagonist), and high dose corticosteroids. The discussion focuses on clinical presentation, pathogenesis and treatment of MAS with an emphasis on MAS secondary to HME. Conclusion Macrophage activation syndrome or sHLH is a dysfunctional, hyperactive and potentially fatal immune system response that results in multi-organ dysfunction. With increasing incidence of Ehrlichia chaffeensis as an emerging pathogen, clinicians should be aware of this fulminant and potentially fatal complication of HME.Indian Journal of Hematology and Blood Transfusion 09/2014; 30(Suppl 1):145-7. DOI:10.1007/s12288-013-0299-3 · 0.23 Impact Factor