Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of Enteropathy-Associated T-Cell Lymphoma

Varghese, Dona; Koya, Hayas Haseer; Cherian, Sujith V.; Mead, Kristen; Sharma, Amit; Sharma, Namita; Knohl, Stephen J.; Benjamin, Sam

doi:10.1200/jco.2012.43.4944

Cited by 10 publications

(5 citation statements)

References 24 publications

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“…Diarrhoea and bowel symptoms are not part of the formal HLH 2004 or HLH 2008 diagnostic criteria, and as a result, the presence of diarrhoea in HLH/MAS is not well-described in the literature 11. A handful of case reports have noted an association between HLH and diarrhoea secondary to various abdominal pathologies such as appendicitis, IBD, infectious colitis and T-cell lymphoma 38–42. Pagel et al identified a subtype of inherited familial HLH (type 5 familial HLH) presenting with copious, severe, chronic watery diarrhoea, in which defects in the lysosomal-dependent exocytosis pathway (STXBP2) were inherited in an autosomal recessive manner 43.…”

Section: Discussionmentioning

confidence: 99%

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

et al. 2022

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Haemophagocytic lymphocytic histiocytosis (HLH) is a rare, life-threatening condition caused by abnormal activation of cytotoxic T lymphocytes, natural killer cells and macrophages resulting in hypercytokinaemia and immune-mediated injury of multiple organ systems. Secondary HLH occurs in the setting of a malignant, infectious or autoimmune stimulus. Macrophage activation syndrome (MAS) is the term used to describe HLH that develops secondary to rheumatological diseases such as lupus and juvenile idiopathic arthritis, among others. Commonly observed and documented symptoms include fever, organomegaly and lymphadenopathy. Given the potential for multiorgan failure in HLH/MAS, early identification, diagnosis and initiation of treatment is essential. We present a case of secondary HLH/MAS with acute inflammatory gastroenteritis in a middle-aged woman with a history of systemic lupus erythematosus.

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Section: Discussionmentioning

confidence: 99%

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

et al. 2022

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“…11 HLH can be an uncommon and usually fatal complication of EATL. 13 It has been reported that six patients with EATL-related haemophagocytic syndrome died within 3 months after the appearance of haemophagocytic syndrome. 14…”

Section: Discussionmentioning

confidence: 99%

“…11 HLH can be an uncommon and usually fatal complication of EATL. 13 It has been reported that six patients with EATLrelated haemophagocytic syndrome died within 3 months after the appearance of haemophagocytic syndrome. 14 In this current case, the patient's condition rapidly deteriorated into HLH after the patient discontinued the GFD.…”

Section: Discussionmentioning

confidence: 99%

A fatal outcome in a patient with coeliac disease who suspended a strict gluten-free diet: a case report

Zheng

Hua

et al. 2021

J Int Med Res

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Coeliac disease (CD) is an autoimmune small bowel disease that occurs in susceptible individuals that develop an immunological reaction to gluten. A strict gluten-free diet (GFD) is the primary treatment for CD. This case report describes a patient with CD recurrence due to a discontinuation of a strict GFD by the patient. After recurrence, the patient developed fever and pancytopaenia, and quickly died of haemophagocytic lymphohistiocytosis (HLH). To the best of our knowledge, this is the first description of a case of CD associated with HLH due to discontinued GFD, which may contribute to improving the awareness of the importance of maintaining a strict GFD and having regular follow-up examinations.

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“…B-symptoms (fever, night sweats, fatigue), on the other hand, are uncommon [ 1 ]. Other more rarely reported manifestations include skin lesions due to cutaneous infiltration, ascites, seizures and altered mental status, peripheral nerve infiltration, obstructive jaundice, bilateral ovarian masses, renal failure due to kidney infiltration, pleural mass, infiltrative cardiomyopathy, eosinophilia, autoimmune hemolytic anaemia and hemophagocytic lymphohistiocytosis (HLH) [ 18 – 31 ].…”

Section: Discussion/literature Reviewmentioning

confidence: 99%

Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges

Sánchez

Qian

Barbieri

et al. 2017

ecancer

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Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), previously known as enteropathy associated T-cell lymphoma (EATL) type II, is a rare haematological malignancy with a difficult and delayed diagnosis. Symptoms can include abdominal pain, weight loss, and chronic diarrhoea. However, most patients are only diagnosed after complications, such as perforation or obstruction, have developed. There is no standard treatment for MEITL; most accepted regimens consist of surgical resection and anthracycline-based chemotherapy. Prognosis is poor with an approximate survival of less than a year. Even though other therapies, such as autologous stem cell transplant, has shown promising results, not all patients can tolerate this course of treatment especially if they are elderly, have several comorbidities or are malnourished. Innovative therapies that improve survival and can be used as an alternative for more intensive treatment are needed. We report the use of PEG-asparaginase along with conventional anthracycline therapy in a 70-year-old woman diagnosed with MEITL, who went into remission and survived for more than one year before succumbing to relapsed disease.

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Hemophagocytic Lymphohistiocytosis: An Uncommon Presentation of Enteropathy-Associated T-Cell Lymphoma

Cited by 10 publications

References 24 publications

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

Haemophagocytic lymphocytic histiocytosis/macrophage activation syndrome with acute inflammatory gastroenteritis

A fatal outcome in a patient with coeliac disease who suspended a strict gluten-free diet: a case report

Use of PEG-asparaginase in monomorphic epitheliotropic intestinal T-cell lymphoma, a disease with diagnostic and therapeutic challenges

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