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Akimune Kaga, Hiroshi Watanabe, Hiroki Miyabayashi, Takaya Metoki, Setsuko Kitaoka, Satoru Kumaki
Neonatal toxic shock syndrome-like exanthematous disease (NTED) is a newly recognized neonatal infectious disease, caused by the superantigen toxic shock syndrome toxin-1 (TSST-1). TSST-1 is mainly produced by methicillin-resistant Staphylococcus aureus, and the immune responses to TSST-1 are known to cause toxic shock syndrome, a life-threatening infectious disease. The clinical symptoms of NTED are skin rash, fever, and thrombocytopenia, but severe thrombocytopenia is rare in term infants with NTED. Although the cause of NTED is the same as that of toxic shock syndrome, the clinical symptoms of NTED are milder than toxic shock syndrome...
2016: Tohoku Journal of Experimental Medicine
Kriselle Lao, Namita Sharma, Ajeet Gajra, Neerja Vajpayee
OBJECTIVE: The purpose of this study was to correlate the significance of bone marrow hemophagocytosis and analyze outcome data in patients with suspected hemophagocytic lymphohistiocytosis (HLH) at a tertiary care hospital during the course of 5 years. METHODS: The pathology database of State University of New York Upstate Medical University, Syracuse, was searched for the terms "hemophagocytosis," "hemophagocytic syndrome," and "hemophagocytic lymphohistiocytosis" encompassing the period January 2009-December 2014...
October 2016: Southern Medical Journal
Mark Wunderlich, Courtney Stockman, Mahima Devarajan, Navin Ravishankar, Christina Sexton, Ashish R Kumar, Benjamin Mizukawa, James C Mulloy
Transgenic expression of key myelosupportive human cytokines in immune-deficient mice corrects for the lack of cross-species activities of stem cell factor (SCF), IL-3, and GM-CSF. When engrafted with human umbilical cord blood (UCB), these triple-transgenic mice produce BM and spleen grafts with much higher myeloid composition, relative to nontransgenic controls. Shortly after engraftment with UCB, these mice develop a severe, fatal macrophage activation syndrome (MAS) characterized by a progressive drop in rbc numbers, increased reticulocyte counts, decreased rbc half-life, progressive cytopenias, and evidence of chronic inflammation, including elevated human IL-6...
September 22, 2016: JCI Insight
Nicholas J Fordham, Richa Ajitsaria, Leena Karnik, Subarna Chakravorty
BACKGROUND: This is the first documented case of a patient with hemophagocytic lymphohistiocytosis in association with coeliac disease. There was complete clinical and biochemical remission of hemophagocytic lymphohistiocytosis following the introduction of a gluten-free diet. CASE PRESENTATION: A 7-year-old white girl presented with fevers and maculopapular rash with a recent history of tonsillitis. Blood tests revealed thrombocytopenia (64×10(9)/L), anemia (80 g/L), hypofibrinogenemia (1 g/L), and hyperferritinemia (71,378 μg/L)...
2016: Journal of Medical Case Reports
Amy Cheng, Feifei Williams, James Fortenberry, Catherine Preissig, Steven Salinas, Pradip Kamat
Ehrlichiosis, caused by transmission of Ehrlichia chaffeensis to humans through the bite of an infected lone star tick, can lead to secondary hemophagocytic lymphohistiocytosis (HLH), a life-threatening condition caused by uncontrolled activation of the cellular immune system. We describe a child with HLH secondary to ehrlichiosis who developed multiorgan failure and was successfully managed with extracorporeal membrane oxygenation (ECMO). A 9-year-old boy developed headaches, fever, and sore throat after suspected tick exposure...
September 15, 2016: Pediatrics
Alina Nicolae, Karthik A Ganapathi, Trinh Hoc-Tran Pham, Liqiang Xi, Carlos A Torres-Cabala, Nahid M Nanaji, Hongbin D Zha, Zhen Fan, Sybil Irwin, Stefania Pittaluga, Mark Raffeld, Elaine S Jaffe
Aggressive natural killer cell leukemia (ANKL) is a systemic NK-cell neoplasm, almost always associated with Epstein-Barr virus (EBV). Rare cases of EBV-negative ANKL have been described, and some reports suggested more indolent behavior. We report the clinicopathologic, immunophenotypic, and molecular characteristics of 7 EBV-negative ANKL. All patients were adults, with a median age of 63 years (range 22 to 83 y) and an M:F ratio of 2.5:1. Five patients were White, 1 Black, and 1 Asian. All patients presented acutely, with fever (6/7), cytopenias (6/7), and splenomegaly (4/7)...
September 14, 2016: American Journal of Surgical Pathology
Uroosa Ibrahim, Muhammad N Siddique, Gautam Valecha, Masoud Asgari, Edhan Isaac, Meekoo Dhar
A 46‑year‑old obese male with a medical history of thalassemia minor presented to the emergency room with complaints of severe fatigue and jaundice worsening over two weeks. On further evaluation, the patient was found to have significant hyperbilirubinemia and transaminitis. The hospital course was further complicated by pancytopenia requiring multiple transfusions, worsening hyperbilirubinemia, severe hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia. He was also found to have splenomegaly and evidence of hemophagocytosis on bone marrow biopsy...
2016: Curēus
Norma E González, Silvia Álvarez Ponte, Mariela López, Pablo Fronti, Silvina Smith, Victor Pawluk
The secondary hemophagocytic syndrome is rare in children and even rarer associated with tuberculosis. e report the case of a patient with acquired immunodeficiency syndrome, disseminated tuberculosis and hemophagocytic syndrome. An 8-year-old girl, diagnosed with acquired immunodeficiency syndrome, was admitted due to fever, vomiting and abdominal pain. She presented abdominal distension, dehydration, tachypnea, crackles and wheezing in both lungs, anemia, thrombocytopenia and coagulopathy. She received broad-spectrum antibiotics and exploratory laparotomy was performed with appendectomy and lymph node biopsy...
October 1, 2016: Archivos Argentinos de Pediatría
Vehbi Doğan, Erhan Karaaslan, Samet Özer, Rüveyda Gümüşer, Resul Yılmaz
BACKGROUND: Kawasaki disease is a systemic vasculitis predominately affecting coronary arteries. Hemophagocytic lymphohistiocytosis can complicate the course of Kawasaki disease. Rare cases of secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of Kawasaki disease have been reported. CASE REPORT: We report here a 4 month-old girl with diffuse coronary ectasia and secondary hemophagocytic lymphohistiocytosis occurring during the acute phase of incomplete Kawasaki disease...
July 2016: Balkan Medical Journal
Shino Magaki, Nora Ostrzega, Elliot Ho, Catherine Yim, Phillis Wu, Harry V Vinters
Hemophagocytic lymphohistiocytosis (HLH) is a rare immune hyperactivation syndrome which may be primary (genetic) or secondary to various immune-related conditions including infection, immunodeficiency, and malignancies. Rapid diagnosis and treatment is essential as it can be associated with significant morbidity and mortality. Epstein-Barr virus (EBV) is a known infectious cause of acquired HLH, but EBV-associated HLH involving the central nervous system (CNS) is rare and not well characterized neuropathologically...
August 26, 2016: Human Pathology
Ghulam Rehman Mohyuddin, Heather J Male
Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome characterized by excessive activation of the immune system. Bacterial infections are very rare precipitants of this disease. A 19-year-old gentleman presented with headache, fatigue, and malaise. He was found to be hypotensive, tachycardic, and febrile. Broad spectrum antibiotics were initiated, and a lumbar puncture ruled out meningitis. Patient progressively developed shock that required use of vasopressors, as well as renal and respiratory failure...
2016: Case Reports in Hematology
E Scott Halstead, Surender Rajasekaran, Julie C Fitzgerald, Scott L Weiss
We describe a case of an infant with HSV meningitis and septic shock who demonstrated a remarkably high serum ferritin level. Aggressive pediatric intensive care and the administration of high-dose glucocorticoids were not able to reverse the multiple organ dysfunctions. Subsequent autopsy identified the presence of hemophagocytosis, thus the patient fulfilled hemophagocytic lymphohistiocytosis (HLH) criteria post-mortem. This case highlights that serum ferritin may be an important early indicator of mortality in sepsis due to a cytokine storm similar to macrophage activation syndrome and HLH...
2016: Frontiers in Pediatrics
Chien-Chin Chen, Kung-Chao Chang, L Jeffrey Medeiros, Julia Yu-Yun Lee
BACKGROUND: Hydroa vacciniforme (HV) is associated with Epstein-Barr virus (EBV) infection and a risk of transformation to lymphoma. METHODS: We retrospectively analyzed 6 HV cases for EBV association and transformation to HV-like T-cell lymphoma. Clinicopathologic features were reviewed and cases were assessed for EBV-encoded RNA (EBER) by in situ hybridization, double staining with immunohistochemistry and EBER, and for T-cell clonality. RESULTS: The male-to-female ratio was 5:1, with a median age at diagnosis of 18...
August 17, 2016: Journal of Cutaneous Pathology
Satoru Nanno, Hideo Koh, Takako Katayama, Masamichi Hashiba, Ayumi Sato, Yosuke Makuuchi, Joji Nagasaki, Masatomo Kuno, Takuro Yoshimura, Hiroshi Okamura, Mitsutaka Nishimoto, Asao Hirose, Mika Nakamae, Takahiko Nakane, Masayuki Hino, Hirohisa Nakamae
Objective Recent studies suggest that presepsin (soluble CD14-subtype) is a useful diagnostic and prognostic marker for sepsis, with secretion by activated macrophages potentially dependent on phagocytosis of microorganisms. As "hemophagocytosis" is one of the major characteristics in patients with hemophagocytic syndrome (HPS), we hypothesized that presepsin may reflect the phagocytic activity and be a useful prognostic marker for HPS. Therefore, we aimed to assess the prognostic potential of presepsin in secondary HPS in adult patients with hematological malignancies...
2016: Internal Medicine
Kota Yoshifuji, Takahiro Oshina, Saeko Sonokawa, Yuma Noguchi, Sayaka Suzuki, Keisuke Tanaka, Takashi Kumagai
A 34-year-old man, working at a park in Tokyo, Japan, was repeatedly bitten by mosquitoes while cutting grass. He was hospitalized with sudden fever, fatigue, and weakness. He was eventually diagnosed with dengue virus infection, detected using reverse transcription polymerase chain reaction for the genome and by the presence of nonstructural protein 1 in his peripheral blood. Symptomatic treatments such as acetaminophen for the fever were not effective. Moreover, peripheral blood examination showed drastically decreased white blood cells and platelets, as well as marked elevations of ferritin and soluble interleukin 2 receptor...
July 2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Ki Sun Jung, Su-Hee Cho, Seok Jin Kim, Young Hyeh Ko, Won Seog Kim
The classification of mature NK-/T-cell lymphoma mainly originating from the T-cell lineage with predominantly nodal involvement and Epstein-Barr virus (EBV) positivity in a majority of tumor cells is unresolved. We analyzed the clinical features and treatment outcomes of such patients. Five patients with EBV-positive nodal T-cell lymphoma were surveyed during follow-up period. The median age was 53 years (range 33-88 years), and all patients showed nodal involvement. The patients mostly presented advanced clinical features, such as stage III or IV disease, elevated lactate dehydrogenase, and hemophagocytosis...
July 25, 2016: International Journal of Hematology
Steven Grangé, Gérard Buchonnet, Emmanuel Besnier, Elise Artaud-Macari, Gaetan Beduneau, Dorothée Carpentier, Julien Dehay, Christophe Girault, Antoine Marchalot, Dominique Guerrot, Fabienne Tamion
OBJECTIVES: Thrombocytopenia is a common, multifactorial, finding in ICU. Hemophagocytosis is one of the main explanatory mechanisms, possibly integrated into hemophagocytic lymphohistiocytosis syndrome, of infectious origin in the majority of cases in ICU. The hemophagocytic lymphohistiocytosis is probably underdiagnosed in the ICU, although it is associated with dramatic outcomes. The main objectives of this work were to identify the frequency of secondary hemophagocytic lymphohistiocytosis, and the main prognostic factors for mortality...
November 2016: Critical Care Medicine
Aradhana Harrison, Dinesh Chandra, Naveen Kakkar, Sheila Das, M Joseph John
Hemophagocytosis shows engulfment of hematopoietic cells by histiocytes and is a property generally associated with cells of the histiocytic lineage. It can be familial or is seen in a wide spectrum of acquired disorders. Hemophagocytosis by leukemic blasts is an uncommon phenomenon and has been reported mainly in acute myeloid leukemia. Its association with acute lymphoblastic leukemia is rare. We present a case of hemophagocytosis by blasts in the bone marrow in a 11 year old boy with T cell-acute lymphoblastic leukemia...
June 2016: Indian Journal of Hematology & Blood Transfusion
María Teresa Fernández-Figueras, María Teresa Martín-Urdà, Adrià Plana, Octavi Servitje, Rosa María Penin, Gustavo Tapia, José Luis Mate, Aurelio Ariza
AIMS: Many types of intravascular lymphohistiocytic proliferation have been lately described. This was an unnoticed or misinterpreted phenomenon, until recently. Intralymphatic lymphohistiocytic aggregates are relatively common and include benign, malignant, and indeterminate conditions. In contrast, all non-endothelial proliferations in the lumina of blood vessels have been interpreted as malignant so far. Herein, we present three cases of histiocytic proliferations in the lumen of blood vessels associate to extensive hemophagocytosis a previously undescribed entity...
July 5, 2016: Histopathology
Asma Bandhani, Naila Raza, Kamal Ahmed
Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome seen primarily in children. It is characterized by pathologic systemic hyper inflammation which in adults is easily overlooked due to non-specific clinical features. Most of the data available are on paedriatic population, making the diagnosis of HLH in adults challenging for the clinician. Here we report a case of HLH in a 48-year male who presented with pyrexia of unknown origin for 2 months but remained undiagnosed despite extensive workup...
June 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
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