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Hemophagocytosis

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https://www.readbyqxmd.com/read/28024491/-children-s-nk-t-cell-lymphoma-associated-hemophagocytic-syndrome-clinical-analysis-of-6-cases
#1
Yong-Zhi Zheng, Hao Zheng, Jian Li, Shao-Hua LE, Xue-Ling Hua, Zai-Sheng Chen, Ling Zheng, Mei Li, Yi-Qiao Chen, Qin-Li Gao, Jing-Hui Yang, Jian-Da Hu
OBJECTIVE: To study the clinical features, treatment and prognosis of patients with NK/T cell lymphoma-associated hemophagocytic syndrome(NK/T-LAHPS). METHODS: Retrospective analysis was used to explore the clinical data of 6 children with NK/T-LAHPS who were admitted in Department of Pediatric Hematology of Fujian Medical University Union Hospital from July 2012 to June 2016. The 6 patients included 4 boys and 2 girls, with a median age of 4 years(range 1.75 to 11)...
December 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27998299/rituximab-etoposide-methylprednisolone-high-dose-cytarabine-and-cisplatin-in-the-treatment-of-secondary-hemophagocytic-lymphohistiocytosis-with-classical-hodgkin-lymphoma-a-case-report-and-review-of-the-literature
#2
Steve Hu, Pranshu Bansal, David Lynch, Cristhiam Mauricio Rojas Hernandez, Zoneddy Dayao
BACKGROUND: Hemophagocytic lymphohistiocytosis is becoming an increasingly recognized disorder in adults. Classical Hodgkin lymphoma is a relatively uncommon etiology of hemophagocytic lymphohistiocytosis and may complicate treatment options. Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin are discussed here as a treatment regimen. CASE PRESENTATION: A 66-year-old Hispanic man previously in good health presented with a 1-month history of recurrent fevers, chills, and night sweats and a 3-week history of new onset jaundice...
December 20, 2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27990393/subcutaneous-panniculitis-like-t-cell-lymphoma-with-macrophage-activation-syndrome-treated-by-cyclosporine-and-prednisolone
#3
Dinesh P Asati, Vaibhav Ingle, Deepti Joshi, Anurag Tiwari
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL; α/β T-cell subtype) is a distinct variantof cutaneous T-cell lymphomas, which presents as inflammatory subcutaneous nodules. A 17-year-old male presented with recurrent fever with concomitant facial swelling, pedal edema, hepatosplenomegaly, and mildly tender subcutaneous plaques in generalized distribution along with patches of scarring alopecia on scalp. There were features of macrophage activation syndrome in the form of hemophagocytosis in the bone marrow, pancytopenia, high serum lactate dehydrogenase levels, low fibrinogen clotting activity, prolonged activated prothrombine time (aPTT), increased serum ferritin, hypoalbuminemia, and hypertriglyceridemia...
November 2016: Indian Dermatology Online Journal
https://www.readbyqxmd.com/read/27922757/hemophagocytic-lymphohistiocytosis-triggered-by-gaucher-disease-in-a-preterm-neonate
#4
Simone Schüller, Andishe Attarbaschi, Angelika Berger, Caroline Hutter, Katrin Klebermass-Schrehof, Manuel Steiner
OBJECTIVE: To present the diagnostic workup in an extremely low birth weight infant patient with signs of both sepsis and hemophagocytosis. PARTICIPANTS: A preterm infant presented with clinical and laboratory signs of early-onset sepsis including hepatosplenomegaly, thrombocytopenia, direct hyperbilirubinemia, and elevated liver enzymes. METHODS: Despite extensive septic workup, no underlying infection was detected. Additional hyperferritinemia and other elevated inflammatory parameters raised the suspicion of a primary or secondary hemophagocytic lymphohistiocytosis (HLH)...
October 2016: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/27914778/familial-haemophagocytosis-lymphohisticytosis-type-3-a-case-report
#5
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
https://www.readbyqxmd.com/read/27906695/metformin-induced-generalized-fixed-drug-eruption-with-cutaneous-hemophagocytosis
#6
Jose Luis Ramírez-Bellver, Joaquin Lopez, Elena Macias, Laura Fuertes, Irene Andres, Victoria Alegria, Ignacio Gimeno, Alejandra Perez, Yosmar Perez, Luis Requena
Fixed drug eruption (FDE) consists of recurrent dusky-red to brownish macules or patches at the same sites after the readministration of the causative drug. It usually presents as a solitary lesion, but generalized eruptions have been described. The most frequently implied drugs are antibiotics, anticonvulsants, and analgesics. Only 2 cases due to metformin have been reported. Histopathologic features of FDE include vacuolar degeneration of the basal layer, necrotic keratinocytes, and superficial and deep perivascular lymphocytic infiltrate...
December 19, 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27883370/epstein-barr-virus-associated-hemophagocytic-syndrome-after-scrub-typhus-infection
#7
Jeong Woo Hong, Hyun Seon You, Tae Won Lee, Won Yong Jo, Bo Ra Kim, Young Sun Suh, In Gyu Bae, Oh Hyun Cho
There have been a small number of cases of scrub typhus-associated hemophagocytic syndrome (HPS), most of which were treated successfully using adequate antibiotics. Here, we report a case of Epstein-Barr virus (EBV)-associated HPS after scrub typhus infection that was not improved using antirickettsial treatment. A 73-year-old male who had been diagnosed with scrub typhus according to an eschar and a positive serology was transferred to our institution because of a persistent fever despite 7-day doxycycline therapy...
December 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27863755/identification-of-the-myst3-crebbp-fusion-gene-in-infants-with-acute-myeloid-leukemia-and-hemophagocytosis
#8
Francianne Gomes Andrade, Elda Pereira Noronha, Rosania Maria Baseggio, Teresa Cristina Cardoso Fonseca, Bruno Marcelo Rocha Freire, Isis M Quezado Magalhaes, Ilana R Zalcberg, Maria S Pombo-de-Oliveira
BACKGROUND: Acute myeloid leukemia presenting the MYST3-CREBBP fusion gene is a rare subgroup associated with hemophagocytosis in early infancy and monocytic differentiation. The aim of this study was to define the relevant molecular cytogenetic characteristics of a unique series of early infancy acute myeloid leukemia cases (≤24months old), based on the presence of hemophagocytosis by blast cells at diagnosis. METHODS: A series of 266 infant cases of acute myeloid leukemia was the reference cohort for the present analysis...
October 2016: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/27847518/adult-onset-still-s-disease-associated-with-mycoplasma-pneumoniae-infection-and-hemophagocytic-lymphohistiocytosis
#9
Abhishek Agnihotri, Allison Ruff, Lauren Gotterer, Addie Walker, Amy H McKenney, Andrei Brateanu
Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated...
2016: Case Reports in Medicine
https://www.readbyqxmd.com/read/27846765/adult-onset-still-s-disease-like-manifestation-accompanied-by-the-cancer-recurrence-after-long-term-resting-state
#10
Kazuhito Fukuoka, Ayako Miyamoto, Yuko Ozawa, Noriko Ikegaya, Tomohiro Maesono, Yoshinori Komagata, Shinya Kaname, Yoshihiro Arimura
A 72-year-old woman presented 9 months ago with skin rash on her bilateral forearms, which was followed by intermittent high fever, and stiffness and swelling of her bilateral fingers. She was diagnosed with seronegative rheumatoid arthritis (RA). She had a past history of breast cancer and had undergone breast preservation surgery 13 years previously. During admission in our hospital, she developed high fever and leukocytosis with a relapsing skin rash, sore throat, polyarthralgia and increased levels of serum ALT/AST and ferritin, all of which fulfilled Yamaguchi's criteria for adult-onset Still's disease (AOSD)...
December 9, 2016: Modern Rheumatology
https://www.readbyqxmd.com/read/27826329/recurrent-macrophage-activation-syndrome-since-toddler-age-in-an-adolescent-boy-with-hla-b27-positive-juvenile-ankylosing-spondylitis
#11
Joon Hyeong Park, Yu Mi Seo, Seung Beom Han, Ki Hwan Kim, Jung Woo Rhim, Nack Gyun Chung, Myung Shin Kim, Jin Han Kang, Dae Chul Jeong
Recurrent macrophage activation syndrome (MAS) is very rare. We present the case of an adolescent boy with human leukocyte antigen (HLA) B27-positive ankylosing spondylitis (AS), who experienced episodes of recurrent MAS since he was a toddler. A 16-year-old boy was admitted because of remittent fever with pancytopenia and splenomegaly after surgical intervention for an intractable perianal abscess. He had been diagnosed with hemophagocytic lymphohistiocytosis (HLH) 4 different times, which was well controlled with intravenous immunoglobulin and steroids since the age of 3...
October 2016: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/27822583/prevalence-severity-and-pathogeneses-of-anemia-in-visceral-leishmaniasis
#12
REVIEW
Yasuyuki Goto, Jingjie Cheng, Satoko Omachi, Ayako Morimoto
Anemia is a typical symptom during visceral leishmaniasis (VL). We performed a systematic analysis of the literature on anemia in VL to understand the prevalence, severity, and possible mechanisms. Anemia is very common in VL patients with an overall prevalence higher than 90 %. The degree of anemia in VL is moderate to severe (hemoglobin level ∼7.5 g/dl), and the status can be recovered by treatment with antileishmanial drugs within a certain period of time. Possible pathogeneses of anemia in VL based on clinical observations included anti-RBC antibodies, dysfunction in erythropoiesis, and hemophagocytosis in the bone marrow or spleen, while hemolysis is a more likely cause than dyserythropoiesis...
November 7, 2016: Parasitology Research
https://www.readbyqxmd.com/read/27818826/macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-successfully-treated-with-anakinra
#13
Aswini Kumar, Hiroshi Kato
Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27803821/hemophagocytic-lymphohistiocytosis-in-a-patient-with-classical-hodgkin-lymphoma
#14
G Hyun, K J Robbins, N Wilgus, L Grosso, S D Goyal
Introduction. Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that can be associated with inherited genetic mutations, malignancy, autoimmune disorders, and viral infections. Though the pathogenesis is not fully known, HLH is understood to be a reactive process in the setting of uncontrolled activation of macrophages, CD8+ cytotoxic lymphocytes, and other immune cells. Hallmark clinicopathological features of HLH include fevers, cytopenias, hepatosplenomegaly, and hemophagocytosis in the bone marrow...
2016: Case Reports in Hematology
https://www.readbyqxmd.com/read/27801337/-the-clinical-characteristics-of-macrophage-activation-syndrome-secondary-to-systemic-lupus-erythematosus
#15
N Jiang, M T Li, D Wu, X F Zeng
Objective: To investigate the clinical features of macrophage activation syndrome (MAS) associated with systemic lupus erythematosus (SLE). Method: The clinical data of 15 patients with SLE-induced MAS diagnosed in Peking Union Medical College Hospital from July 2011 to December 2014 were retrospectively analyzed. Results: Fourteen patients were female. The average age was 28.07. When MAS occurred, the average duration of SLE was 20.47 months, and the average SLE disease activity index (SLEDAI) was 18.4. All 15 patients developed fever, hematocytopenia and impaired liver function in the course of MAS, while patients with splenomegaly, coagulation disorders and neuropsychiatric symptoms were 11, 14 and 8, respectively...
November 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/27795864/histiocytic-sarcoma-in-a-kidney-transplant-patient-a-case-report-and-review-of-the-literature
#16
Maressa Pollen, Siraj El Jamal, Jack Lewin, Varsha Manucha
Objective. Histiocytic sarcoma (HS) is an aggressive neoplasm with only limited number of reported series of cases and rare case reports of occurrence as a posttransplant neoplastic disorder. The etiology and pathogenesis of the disease is unknown and the optimal treatment is still under investigation. We describe an unusual case of HS in a patient with a remote history of kidney transplant. Method and Results. A 54-year-old male with a remote history of renal transplantation under maintenance immunosuppression presented with features of sepsis...
2016: Case Reports in Pathology
https://www.readbyqxmd.com/read/27795540/primary-immunodeficiency-disease-and-hematology
#17
Hirokazu Kanegane
Primary immunodeficiency disease (PID) is an inborn error of the immune system, and is characterized by not only susceptibility to infection but also frequent combination with autoimmune diseases and malignancies. PID is principally caused by a germline mutation, and some PID patients develop hematological abnormalities. In some patients, PID is associated with hemophagocytosis-induced cytopenia, neutropenia, thrombocytopenia, and autoimmune cytopenia. In addition, a subset of PID patients presented with myeloid dysplasia...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27785117/a-retrospective-analysis-of-56-children-with-hemophagocytic-lymphohistiocytosis
#18
Yan-Rong Wang, Yi-Ning Qiu, Yan Bai, Xian-Feng Wang
AIM: The aim of this study was to investigate the etiological factors, clinical features, and prognostic factors in children with hemophagocytic lymphohistiocytosis (HLH). METHODS: Fifty-six children with HLH in Wuhan Union Hospital, People's Republic of China, were retrospectively analyzed in recent years. We reviewed the medical records of 56 HLH children hospitalized from 2000 to 2013 to identify the possible prognostic factors. RESULTS: In more than half of the cases (64...
2016: Journal of Blood Medicine
https://www.readbyqxmd.com/read/27776187/epidemiological-and-clinical-features-of-severe-fever-with-thrombocytopenia-syndrome-in-japan-2013-2014
#19
Hirofumi Kato, Takuya Yamagishi, Tomoe Shimada, Tamano Matsui, Masayuki Shimojima, Masayuki Saijo, Kazunori Oishi
Although severe fever with thrombocytopenia syndrome (SFTS) was first reported from Japan in 2013, the precise clinical features and the risk factors for SFTS have not been fully investigated in Japan. Ninety-six cases of severe fever with thrombocytopenia syndrome (SFTS) were notified through the national surveillance system between April 2013 and September 2014 in Japan. All cases were from western Japan, and 82 cases (85%) had an onset between April and August. A retrospective observational study of the notified SFTS cases was conducted to identify the clinical features and laboratory findings during the same period...
2016: PloS One
https://www.readbyqxmd.com/read/27760897/a-term-infant-of-neonatal-toxic-shock-syndrome-like-exanthematous-disease-complicated-with-hemophagocytic-syndrome
#20
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
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