Hemophagocytic Lymphohistiocytosis during the Postpartum Stage of Pregnancy: A Report of Eight Cases

2018 ◽  
Vol 141 (1) ◽  
pp. 55-60 ◽  
Author(s):  
Yue Song ◽  
Jing-Shi Wang ◽  
Yi-Ni Wang ◽  
Zhao Wang

Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. However, most of these cases occur during pregnancy. Considering that the periods before and after delivery have different clinical features, we presented the first case series of HLH that presented during the postpartum stage of pregnancy. From these cases, we concluded that postpartum HLH is a common form of pregnancy-related HLH. Patients with postpartum HLH may suffer more complicated pathogenesis. Cytopenia was not as common as in other types of HLH, but liver dysfunction was present in almost all cases. The standard therapy of HLH-94/04 was very effective, and the outcomes of postpartum HLH were better.

2020 ◽  
Vol 12 (04) ◽  
pp. 281-284
Author(s):  
Sawsan A. Mohammed ◽  
Azza A. Abdelsatir ◽  
Mohamed Abdellatif ◽  
Suliman Hussein Suliman ◽  
Omer Mohammed Ibrahim Elbasheer ◽  
...  

AbstractsBasidiobolomycosis is a fungal infection caused by Basidiobolus ranarum which affects the skin and subcutaneous tissue and rarely the gastrointestinal tract. We report seven cases of gastrointestinal basidiobolomycosis with interesting clinical, radiological, and histological presentations. To our knowledge, this is the first case series of abdominal basidiobolomycosis to be reported from Sudan.


Nutrients ◽  
2021 ◽  
Vol 13 (2) ◽  
pp. 561
Author(s):  
Luca Dalle Carbonare ◽  
Monica Mottes ◽  
Maria Teresa Valenti

Osteonecrosis of the jaw (ONJ) is a severe clinical condition characterized mostly but not exclusively by an area of exposed bone in the mandible and/or maxilla that typically does not heal over a period of 6–8 weeks. The diagnosis is first of all clinical, but an imaging feedback such as Magnetic Resonance is essential to confirm clinical suspicions. In the last few decades, medication-related osteonecrosis of the jaw (MRONJ) has been widely discussed. From the first case reported in 2003, many case series and reviews have appeared in the scientific literature. Almost all papers concerning this topic conclude that bisphosphonates (BPs) can induce this severe clinical condition, particularly in cancer patients. Nevertheless, the exact mechanism by which amino-BPs would be responsible for ONJ is still debatable. Recent findings suggest a possible alternative explanation for BPs role in this pattern. In the present work we discuss how a condition of osteomalacia and low vitamin D levels might be determinant factors.


2018 ◽  
Vol 11 (6) ◽  
pp. 553-558
Author(s):  
Olivier Wijers ◽  
Rosa C. Looijen ◽  
Jens A. Halm ◽  
Tim Schepers

Background: Peripheral fractures of the talus are uncommon. Almost all the literature regarding talar fractures consists of central intra-articular fractures, whereas studies about peripheral talar fractures are lacking. The aim of this study is to increase awareness in diagnosing an unusual peripheral extra-articular medial impression fracture of the talus. Methods: This study includes a retrospective case series of patients with an extra-articular medial impression fracture of the talus. Patient characteristics, trauma mechanism, diagnostics, fracture characteristics, and treatment were reported. Results: Eight consecutive patients with an extra-articular medial impression fracture of the talus were identified. In 80%, the trauma mechanism was a supination or inversion injury of the ankle and foot. An X-ray was obtained in all patients; in 7 (88%) patients, a computed tomography scan was done, and an additional magnetic resonance imaging was done in 3 (38%) patients. In 4 patients (50%), the correct diagnosis was missed at first presentation. The delay between injury and diagnosis was 0 to 180 days (of 36 days on average). Conclusion: This is the first case series to describe patients with a peripheral extra-articular medial impression fracture of the talus. Good clinical examination and judicious use of diagnostic imaging are a necessity to find the talar impression fractures in a timely manner, and treatment can be started immediately. Levels of Evidence: Level V


2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Veeraraghavan Meyyur Aravamudan ◽  
Chaozer Er ◽  
Ikram Hussain ◽  
Nicholas wong wai Cheong ◽  
Chong Chern Hao ◽  
...  

Hemophagocytic lymphohistiocytosis (HLH) is rare and life-threatening medical emergency. Parvovirus infection is rarely associated with HLH. We report a case of parvovirus-related HLH in a patient with alpha thalassaemia (HbH disease). The patient responded well to a course of dexamethasone without the need of etoposide. Based on our literature search, this is the first case of parvovirus related HLH in a patient with HbH disease in the medical literature.


2021 ◽  
Author(s):  
Mohammad Hasan ◽  
Khaled Al Zubaidi ◽  
Karim Diab ◽  
Yahia Hejazi ◽  
Sharon Bout-Tabaku ◽  
...  

Abstract Background: Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe complication of coronavirus disease 2019 (COVID-19) in children, which is increasingly being reported worldwide. Here we report the first case series of 7 children diagnosed with MIS-C in Qatar. Methods: Clinical features and outcomes of COVID-19 positive patients admitted to Sidra Medicine, Qatar from June to October 2020, who met the WHO case definition for MIS-C were reviewed.Results: The mean age in our case series was 5.6 years, of which 71.4% were males. All patients were previously healthy but had a history of COVID-19 infection. Fever, rash, vomiting and abdominal pain were the most common symptoms (70%-100%). The average hospitalization was 12.9 days with no case fatalities. Laboratory findings included lymphopenia and thrombocytopenia in most patients, as well as evidence of coagulopathy and elevated inflammatory markers such as C-reactive protein, ferritin and procalcitonin. Many patients (71.4%) required inotropic support in intensive care, while only one required respiratory support. Although all patients had elevated cardiac biomarkers, cardiovascular involvement was observed in 42.9% of patients with one patient developing a giant coronary aneurysm. All patients received intravenous immunoglobulin (IVIG) and 86% of patients received corticosteroids, with two patients requiring treatment with IL-1 inhibitors.Conclusions: Our report is one of the first reports on MIS-C from Asia. Although clinical features and outcomes are not significantly different from those reported elsewhere, lack of case fatalities in our cohort may indicate that early recognition and prompt medical attention is necessary for a favorable outcome in MIS-C.


2019 ◽  
Vol 42 (11) ◽  
pp. 658-664 ◽  
Author(s):  
Silvius Frimmel ◽  
Michael Hinz ◽  
Jan Schipper ◽  
Simon Bogdanow ◽  
Steffen Mitzner ◽  
...  

Hemophagocytic lymphohistiocytosis is a life-threatening clinical syndrome caused by severe hypercytokinemia brought on by a highly stimulated but ineffective immune response. Animal studies and case series have demonstrated that a reduction in blood cytokine levels achieved with an extracorporeal adsorption cartridge that contains blood-compatible porous polymer beads (CytoSorb®) can effectively attenuate the inflammatory response during sepsis and possibly improve outcomes. We report a case series of two patients in which three episodes of severe hemophagocytic lymphohistiocytosis triggered by infections with herpesviridae were treated successfully with cytokine adsorption. A marked decrease in interleukin-6 plasma levels and a stable or decreasing need of vasopressor therapy were the most significant results of this treatment. Importantly, treatment was safe and well-tolerated, without any adverse events.


2020 ◽  
Vol 21 (2) ◽  
pp. 123-126
Author(s):  
Quazi Tarikul Islam ◽  
Hirinmoy Barman Sagor ◽  
Tasmina Chowdhury Tuli

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening medical condition characterized by hyperphagocytosis secondary to an inappropriate over-activation of macrophages and lymphocytes that driven by excessive cytokines production which resulted in cellular destructions. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. It should be suspected with prolonged fever beyond seven days associated with splenomegaly, hyperferritinemia, worsening cytopenias and development of multiorgan dysfunction. A proportion of patients recovered with supportive therapy, however most required interventions with corticosteroids, intravenous immunoglobulin or chemotherapy. We report 3 cases of dengue associated HLH . Among them 2 patients were treated with steroid with good outcome, and one died from MODS. J MEDICINE JUL 2020; 21 (2) : 123-126


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mohammad Rubayet Hasan ◽  
Khaled Al Zubaidi ◽  
Karim Diab ◽  
Yahia Hejazi ◽  
Sharon Bout-Tabaku ◽  
...  

Abstract Background Multisystem Inflammatory Syndrome in Children (MIS-C) is a severe complication of coronavirus disease 2019 (COVID-19) in children, which is increasingly being reported worldwide. Here we report the first case series of 7 children diagnosed with MIS-C in Qatar. Methods Clinical features and outcomes of COVID-19 positive patients admitted to Sidra Medicine, Qatar from June to October 2020, who met the WHO case definition for MIS-C were reviewed. Results The mean age in our case series was 5.6 years, of which 71.4% were males. All patients were previously healthy but had a history of COVID-19 infection. Fever, rash, vomiting and abdominal pain were the most common symptoms (70–100%). The average hospitalization was 12.9 days with no case fatalities. Laboratory findings included lymphopenia and thrombocytopenia in most patients, as well as evidence of coagulopathy and elevated inflammatory markers such as C-reactive protein, ferritin and procalcitonin. Many patients (71.4%) required inotropic support in intensive care, while only one required respiratory support. Although all patients had elevated cardiac biomarkers, cardiovascular involvement was observed in 42.9% of patients with one patient developing a giant coronary aneurysm. All patients received intravenous immunoglobulin (IVIG) and 86% of patients received corticosteroids, with two patients requiring treatment with IL-1 inhibitors. Conclusions Our report is one of the first reports on MIS-C from Asia. Although clinical features and outcomes are not significantly different from those reported elsewhere, lack of case fatalities in our cohort may indicate that early recognition and prompt medical attention is necessary for a favorable outcome in MIS-C.


2015 ◽  
Vol 7 (3) ◽  
Author(s):  
Bachar Samra ◽  
Mohamad Yasmin ◽  
Sami Arnaout ◽  
Jacques Azzi

Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses accompanying this syndrome further complicate the problem. A pronounced hyperferritinemia in the setting of systemic signs and symptoms along with a negative infectious and rheumatological workup should raise suspicions for HLH. While treatment ideally consists of immunosuppressive chemotherapy and hematopoietic stem cell transplant, the potential toxicity to both the pregnant woman and the fetus poses a challenging decision. We report the first case of idiopathic HLH presenting as fever of unknown origin in a pregnant woman successfully treated with steroids.


2008 ◽  
Vol 14 (5) ◽  
pp. 671-678 ◽  
Author(s):  
Q Cheng ◽  
L Miao ◽  
J Zhang ◽  
YT Guan ◽  
ZG Liu ◽  
...  

Objective To describe clinical features of patients with multiple sclerosis (MS) in Shanghai, China. Methods Prevalent patients with MS were identified and investigated by a network of physicians in 11 districts of Shanghai during the period from 1 September 2004 to 31 August 2005. Admission registries of each hospital in the study area were checked systematically for patients with a diagnosis of MS, neuromyelitis optica or other demyelinating disorders. All patients with collected information were evaluated by four senior neurologists according to the McDonald criteria. Results There were 249 (146 female and 103 male) patients with a confirmed MS diagnosis, at a female-to-male ratio of 1.4. The mean age at onset of MS was 37.4 years for the 249 patients with MS and, on the prevalence day, 42.7 years. The most frequent location of clinical MS lesions in the central nervous system was the spinal cord (61%), followed by the cerebrum (55%) and optic nerves (41%). Nearly all (96%) of the patients with MS had been examined by magnetic resonance imaging, and 226 (94%) patients of those examined were suggestive of MS. No family history of MS was found in any of the patients. Most (86%) of the patients had no or mild disability on the prevalence day (31 December 2004). Almost all (96%) patients with MS had been treated with corticosteroids. Conclusion Clinical features of patients with MS are described based on the information from the largest case series reported among Chinese. Comparisons and discussions are made with findings from the other populations.


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