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F Kamoun, M Hsairi, V Grandin, S Ben Ameur, G De Saint Basile, M Hachicha
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune regulation. Here, we report on a fatal case of type 3 FHL (FHL3) in a 45-day-old boy. Clinically, the infant presented with fever and hepatosplenomegaly. Biology showed pancytopenia, elevated ferritin, and decreased fibrinogen. Images of hemophagocytosis were found at the bone morrow examination. The diagnosis of FHL type 3 was made by the identification of homozygous mutation in the Munc13-4 gene (UNC13D) located in exon 20: 1822 del 12bp (V608fs)...
January 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
Robin George Manappallil
Macrophage Activation Syndrome (MAS) is a rare form of Haemophagocytic Lymphohistiocytosis (HLH) associated with rheumatologic conditions. It is characterised by haemophagocytosis and cytokine overproduction, resulting from the activation and uncontrolled proliferation of T lymphocytes and macrophages. The patient being reported is an elderly female with Rheumatoid Arthritis (RA), who presented with fever and was found to have MAS. She later developed Acute Respiratory Distress Syndrome (ARDS) and died. Such a scenario has rarely been reported...
September 2016: Journal of Clinical and Diagnostic Research: JCDR
Austen J J Worth, Charlotte J Houldcroft, Claire Booth
Epstein-Barr virus (EBV) infection is ubiquitous in humans, but the majority of infections have an asymptomatic or self-limiting clinical course. Rarely, individuals may develop a pathological EBV infection with a variety of life threatening complications (including haemophagocytosis and malignancy) and others develop asymptomatic chronic EBV viraemia. Although an impaired ability to control EBV infection has long been recognised as a hallmark of severe T-cell immunodeficiency, the advent of next generation sequencing has identified a series of Primary Immunodeficiencies in which EBV-related pathology is the dominant feature...
November 2016: British Journal of Haematology
Rosario M Morales-Camacho, María Díaz-Sánchez, Ricardo Alcántara, Raquel Lamas-Pérez, Fátima de la Cruz Vicente, Emilio Franco-Macías
No abstract text is available yet for this article.
October 2016: British Journal of Haematology
Rosario M Morales-Camacho, Teresa Caballero-Velázquez, Concepción Prats-Martín, Olga Pérez-López, M Teresa Vargas, Estrella Carrillo-Cruz
No abstract text is available yet for this article.
August 2016: British Journal of Haematology
Anupriya Bansal, Suchi Mittal, Jasmita Dass, Nitin Gupta, P K Agarwal, Jyoti Kotwal
De novo CD5+ Diffuse large B cell lymphoma (DLBCL) is a rare and aggressive subtype of DLBCL. It is a distinct clinicopathologic entity with complex molecular profile and poor prognosis. A 59 year old female presented with pyrexia of unknown origin since 1 month. On examination, there was severe pallor, hepatosplenomegaly and no palpable lymphadenopathy. Complete blood count revealed bicytopenia with normal total leucocyte count. Liver and renal function tests were normal. Ultrasonography abdomen revealed splenic enlargement with two focal lesions attributed to either splenic abscess or infarcts...
June 2016: Indian Journal of Hematology & Blood Transfusion
Kamuran Karaman, Sinan Akbayram, Sultan Kaba, Serap Karaman, Mesut Garipardiç, Ilyas Aydin, Ahmet Fayik Öner
This retrospective study included seven paediatric cases aged from 4 to 14 (10.2±3.4) years with pathologically proved haemophagocytic lymphohistiocytosis from a single institution during 2009 and 2013. Over this time period, 496 patients with brucellosis were diagnosed. None of the patients (3 boys and 4 girls) had a history of any haematologic disorder. All patients had an anamnesis for recently consumed unpasteurised homemade dairy products or had a contact history with sheep and/or cows. The diagnosis of brucellosis was confirmed by standard tube agglutination test in all patients; titres were 1: 1280 in seven patients...
June 1, 2016: Le Infezioni in Medicina
Waqas Ullah, Hafez Mohammad Ammar Abdullah, Shayan Qadir, Muhammad Asim Shahzad
Haemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that is caused by an abnormal activation of the immune system. It can present as the primary syndrome or occur secondary to a variety of conditions such as malignancy, autoimmune diseases and infections. We present a case of a man who developed HLH secondary to Plasmodium vivax infection. He presented with symptoms of fever, chills and myalgias. Physical examination revealed significant hepatosplenomegaly. The presence of pancytopaenia, elevated ferritin levels and haemophagocytosis on bone marrow biopsy confirmed the diagnosis of HLH (based on HLH-2004 criteria)...
June 13, 2016: BMJ Case Reports
Marc Gregory Yu, Jamie Chua
Haemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of pathological immune activation characterised by extreme inflammation. We present a case of a young Filipino man consulting for non-specific symptoms of fever, body malaise and weight loss. Prominent physical examination findings included gross pallor, cachexia and hepatosplenomegaly. Laboratory results revealed pancytopaenia, while bone marrow examination revealed haemophagocytosis. Further workup for HLH showed hypertriglyceridaemia, hypofibrinogenaemia and hyperferritinaemia (fulfilling 6 of 8 diagnostic criteria)...
June 9, 2016: BMJ Case Reports
Namhee Kim, Kye-Hyung Kim, Su Jin Lee, Sang-Hyuk Park, In-Suk Kim, Eun Yup Lee, Jongyoun Yi
AIMS: Severe fever with thrombocytopenia syndrome (SFTS) is an emerging tick-borne disease caused by the SFTS virus; primary manifestations are fever, thrombocytopenia, leukopenia and gastrointestinal symptoms. Before an aetiological diagnosis is made, SFTS patients can undergo bone marrow examination due to cytopenias. Although several studies have reported on bone marrow examination in SFTS patients, most do not provide adequate details. Bone marrow findings in SFTS patients were investigated in this study...
June 2016: Journal of Clinical Pathology
Sara Maj Hyldig Matzen, Klaus Kallenbach, Anne Regitze Reumert, Lars Munksgaard
Aggressive NK-cell leukemia is a rare malignancy mostly seen in younger Asians with a rapid clinical course and poor prognosis. Here, we describe a 69 years old Caucasian woman presenting with massive leukemization of neoplastic NK-cells. The cells were abnormal in morphology and surface marker expression and this clearly distinguished them from their normal counterpart. They were large and variable in shapes with irregular folding of the nuclei. By flow cytometry, their light scatter characteristics resembled normal monocytes...
2015: SpringerPlus
Igor Denizarde Bacelar Marques, Renato Antunes Caires, Flávio Jota de Paula, William Carlos Nahas, Elias David-Neto
Haemophagocytic syndrome (HPS) is a rare and potentially lethal condition characterized by pancytopoenia, fever, organomegaly and widespread proliferation of macrophages phagocytosing blood elements. Among the triggers of this syndrome, excessive immunosuppression in a context of acute rejection has been rarely reported, although it might be underdiagnosed. Here, we report the case of a kidney transplant recipient with allograft dysfunction due to chronic antibody-mediated rejection treated with antithymocyte globulin and plasmapheresis...
October 2013: Clinical Kidney Journal
Takahiro Yasumi, Masayuki Hori, Eitaro Hiejima, Hirofumi Shibata, Kazushi Izawa, Hirotsugu Oda, Kouhei Yoshioka, Kenji Nakagawa, Tomoki Kawai, Ryuta Nishikomori, Osamu Ohara, Toshio Heike
Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune dysregulation and is classified as primary or secondary according to the underlying aetiology. The treatment strategies recommended for these two groups differ substantially; however, it is thought to be impossible to predict the underlying causes of HLH using conventional laboratory tests. Recent studies show that serum levels of soluble interleukin-2 receptor (sIL2R) and ferritin are useful for differentiating some forms of HLH...
August 2015: British Journal of Haematology
Alicja Markuszewska-Kuczynska, Monika Klimkowska, Sofie Regenthal, Agnes Bulanda, Cecilia Kämpe Björkvall, Maciej Machaczka
INTRODUCTION: Gaucher cells (GCs), the lipid-laden storage macrophages, are the pathologic hallmark of Gaucher disease (GD). They are typically 20-100 μm in diameter with eccentrically placed nuclei and cytoplasm with characteristic crinkles and striations. A few previous observations have indicated that sometimes GD patients may display morphology of GCs which is different from this classical description. The aim of our study was to explore the morphological polymorphism of GCs in patients with untreated GD type 1 (GD1)...
2015: Folia Histochemica et Cytobiologica
Julie Broe, Anne Falensteen Lauritzen, Per Boye Hansen
A 60-year-old man with chronic lymphocytic leukaemia was admitted to our department with fever and hepatospleno-megaly. Laboratory findings revealed hyperferritinaemia of 40,300 microgram/l and both liver and renal dysfunction. A bone marrow biopsy showed haemophagocytosis consistent with haemophagocytic syndrome. The serology was compatible with acute Cytomegalovirus infection. The patient received therapy with ganciclovir, prednisolone and gamma globulin, and the acute renal failure was treated with haemodialysis...
December 15, 2014: Ugeskrift for Laeger
Delphine Sculier, Thanh Doco-Lecompte, Mathieu Rougemont, Alexandra Calmy
INTRODUCTION: In HIV patients, haemophagocytic syndrome (HPS) may occur in the presence of cancer, concomitant viral infection, HIV primo-infection or at the initiation of highly active antiretroviral therapy (HAART). Hodgkin lymphoma remains a rare cause of HPS. We describe a case of HPS with very high Epstein Barr virus (EBV) load in a HIV patient as initial manifestation of Hodgkin lymphoma. MATERIALS AND METHODS: A 29-year-old HIV positive man, successfully treated with HAART with an undetectable viral load and CD4 cells count of 438/µl, was admitted for high fever of unknown origin...
2014: Journal of the International AIDS Society
Jinkala Sree Rekha, Rakhee Kar, Sajini Elizabeth Jacob, Neelaiah Siddaraju, Bettadpura Shamana Suryanarayana, Molly Mary Thabah, Rakesh Naik, Nanda Kishore Maroju
Extranodal NK/Tcell lymphomas (ENKTL) are rare, aggressive lymphomas. The most common primary site of involvement is the nasal cavity, nasopharynx and paranasal sinuses. The other sites of primary involvement are skin, gastrointestinal tract and testis. Advanced disease can show lymph node, bone marrow and peripheral blood involvement. We report a case of ENKTL of the jejunum, showing peripheral pancytopenia and haemophagocytosis in the bone marrow. The intestine showed multiple intestinal perforations, with evidence of infiltration by lymphoma with coexistent strongyloides infestation...
September 2014: Indian Journal of Hematology & Blood Transfusion
A G Diwan, Rahul Shewale, Shivakumar Iyer, Amit Nisal, Prakhar Agrawa
Macrophage activation syndrome is a potentially life threatening phenomenon characterised by aggressive proliferation of macrophages and T lymphocytes leading to haemophagocytosis of other blood cells and multi organ failure. Here we present a very unusual combination of leptospirosis and scrub typhus infection leading to macrophage activation syndrome. Scrub typhus associated with macrophage activation syndrome has rarely been reported in India. A 40 year old female presented with high grade fever, seizures, bodyache, arthralgia and severe breathlessness...
January 2014: Journal of the Association of Physicians of India
Toni A Nagy, Sarah M Moreland, Corrella S Detweiler
Bacteria harbour both ferrous and ferric iron transporters. We now report that infection of macrophages and mice with a Salmonella enterica Typhimurium strain containing an inactivated feoB-encoded ferrous iron transporter results in increased bacterial replication, compared to infection with wild type. Inactivation of other cation transporters, SitABCD or MntH, did not increase bacterial replication. The feoB mutant strain does not have an intrinsically faster growth rate. Instead, increased replication correlated with increased expression in macrophages of the fepB-encoded bacterial ferric iron transporter and also required siderophores, which capture ferric iron...
September 2014: Molecular Microbiology
Sreeya Das, Pritinanda Mishra, Rakhee Kar, Debdatta Basu
Gelatinous marrow transformation (GMT) or serous atrophy of bone marrow (BM) is a rare disease characterised by focal marrow hypoplasia, fat atrophy, and accumulation of extracellular mucopolysaccharides abundant in hyaluronic acid. This study reviews 11 cases of GMT from South India. Clinical and haematological parameters, BM aspirate, and biopsies of all patients diagnosed with GMT over a period of 7 years were studied. GMT was diagnosed in BM biopsy based on characteristic morphological appearance and was confirmed by alcian blue positive staining pattern at pH levels of 2...
June 2014: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
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