scholarly journals Primary Haemophagocytic Lymphohistiocytosis (Chédiak‐Higashi Syndrome) triggered by acute SARS‐CoV‐2 infection in a 6‐week‐old infant

2021 ◽  
Author(s):  
M Lange ◽  
T Linden ◽  
HL Müller ◽  
AM Flasskuehler ◽  
H Koester ◽  
...  
Keyword(s):  
Haemophagocytic Lymphohistiocytosis ◽  
Chediak Higashi Syndrome

Acute encephalopathy as a primary manifestation of haemophagocytic lymphohistiocytosis

2001 ◽  
Vol 43 (08) ◽  
pp. 555 ◽  
Author(s):  
Matthias Kieslich ◽  
Marilena Vecchi ◽  
Pablo Hernaiz Driever ◽  
Anna Maria Laverda ◽  
Dirk Schwabe ◽  
...  
Keyword(s):  
Acute Encephalopathy ◽  
Haemophagocytic Lymphohistiocytosis

Gene Therapy for Haemophagocytic Lymphohistiocytosis

2014 ◽  
Vol 14 (6) ◽  
pp. 437-446 ◽  
Author(s):  
Claire Booth ◽  
Marlene Carmo ◽  
H. Gaspar
Keyword(s):  
Gene Therapy ◽  
Haemophagocytic Lymphohistiocytosis

Haemophagocytic Lymphohistiocytosis (HLH) in a case of Enteric Fever

2016 ◽  
Vol 5 (03) ◽  
pp. 4882
Author(s):  
Vineeta Pande ◽  
Agarkhedkar S. R. ◽  
Ayank Tandon* ◽  
Aditya Agarwal
Keyword(s):  
Inflammatory Reaction ◽  
Enteric Fever ◽  
Infectious Agent ◽  
Clinical Syndrome ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Life Threatening ◽  
Stimulation Of

HLH is an uncommon, life threatening clinical syndrome cause by a severe hyper inflammatory reaction triggered by an infectious agent. The characteristic symptoms of HLH are due to the persistent stimulation of lymphocytes and histiocytes, leading to hyper-cytokinemia. We report a case of HLH in enteric fever in a13 year old female presenting with fever, lymphadenopathy and pancytopenia due an infection caused by Salmonella.

Chemotherapy-sparing treatment of haemophagocytic lymphohistiocytosis with intravenous immunoglobulins and corticosteroids

2020 ◽  
Vol 13 (5) ◽  
pp. e234490
Author(s):  
Evan C Chen ◽  
Jonathan A Stefely ◽  
Bimalangshu R Dey ◽  
Walter H Dzik
Keyword(s):  
Effective Treatment ◽  
Disease Severity ◽  
Critically Ill ◽  
Intravenous Immunoglobulins ◽  
Current Treatment ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Tailored Therapy ◽  
Fatal Disease ◽  
Therapeutic Challenge

Haemophagocytic lymphohistiocytosis (HLH) can be a rapidly fatal disease. Current treatment in adults is extrapolated from the HLH-2004 protocol that specifies a regimen of etoposide, dexamethasone and cyclosporine. However, HLH presents as a spectrum of disease severity. A therapeutic challenge arises for milder cases where the harms of potent chemotherapy such as etoposide may outweigh its benefit. We present a case of an adult with HLH who developed significant pancytopenia but was otherwise not critically ill and who responded to treatment with a chemotherapy-sparing approach consisting of intravenous immunoglobulins and corticosteroids alone. The case illustrates that tailored therapy may allow effective treatment of the disorder while minimising therapy-related toxicities.

scholarly journals Secondary haemophagocytic lymphohistiocytosis in COVID-19: correlation of the autopsy findings of bone marrow haemophagocytosis with HScore

2021 ◽  
pp. jclinpath-2020-207337
Author(s):  
Claudia Núñez-Torrón ◽  
Ana Ferrer-Gómez ◽  
Esther Moreno Moreno ◽  
Belen Pérez-Mies ◽  
Jesús Villarrubia ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Immune Response ◽  
Immune System ◽  
Multiorgan Failure ◽  
Histological Findings ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Bone Marrow Tissue ◽  
Autopsy Findings ◽  
Specific Finding ◽  
Clinical Records

BackgroundSecondary haemophagocytic lymphohistiocytosis (sHLH) is characterised by a hyper activation of immune system that leads to multiorgan failure. It is suggested that excessive immune response in patients with COVID-19 could mimic this syndrome. Some COVID-19 autopsy studies have revealed the presence of haemophagocytosis images in bone marrow, raising the possibility, along with HScore parameters, of sHLH.AimOur objective is to ascertain the existence of sHLH in some patients with severe COVID-19.MethodsWe report the autopsy histological findings of 16 patients with COVID-19, focusing on the presence of haemophagocytosis in bone marrow, obtained from rib squeeze and integrating these findings with HScore parameters. CD68 immunohistochemical stains were used to highlight histiocytes and haemophagocytic cells. Clinical evolution and laboratory parameters of patients were collected from electronic clinical records.ResultsEleven patients (68.7%) displayed moderate histiocytic hyperplasia with haemophagocytosis (HHH) in bone marrow, three patients (18.7%) displayed severe HHH and the remainder were mild. All HScore parameters were collected in 10 patients (62.5%). Among the patients in which all parameters were evaluable, eight patients (80%) had an HScore >169. sHLH was not clinically suspected in any case.ConclusionsOur results support the recommendation of some authors to use the HScore in patients with severe COVID-19 in order to identify those who could benefit from immunosuppressive therapies. The presence of haemophagocytosis in bone marrow tissue, despite not being a specific finding, has proved to be a very useful tool in our study to identify these patients.

Importance of Morphology in the Era of Molecular Biology: Lesson Learnt from a Case of Chediak–Higashi Syndrome

2021 ◽  
Author(s):  
Ankita Singh ◽  
Ankur Kumar Jindal ◽  
Raviteja Indla ◽  
Praveen Sharma ◽  
Neelam Varma ◽  
...  
Keyword(s):  
Molecular Biology ◽  
Chediak Higashi Syndrome
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