An Unusual Patient with Atypical Hemolytic Uremic Syndrome Who Developed Hemophagocytic Lymphohistiocytosis

Abstract

strains, mainly O157, and has a good prognosis (2, 3). Atypical HUS or diarrhea (-) HUS may be idiopathic, familial or due to a variety of conditions such as therapeutic drug usage (ovulation inhibitors, immunosuppressive agents), various diseases (malignancies, systemic lupus erythematosus), pregnancy and infections (4, 5). It can occur at any age including newborns and it has a frequently recurrent course and poor renal prognosis (1, 4, 5).

Hemophagocytic Lymphohistiocytosis Associated With Hemolytic Uremic Syndrome in a Child: A Case Report and Systematic Literature Review

“…Furthermore, the normal level of ADAMTS 13 in HUS is useful in differentiating HUS from TTP. 3,9 Typical HUS, also known as Shiga toxin-associated HUS, is caused by Shiga toxin-producing pathogens such as enterohemorrhagic E. coli and represents 85% to 90% of cases that are commonly observed in infants and young children. Nonetheless, it has a good prognosis.…”

“…TMA is a histopathologic term used to describe a variety of thrombotic vascular lesions that represents a group of diseases that include HUS, and thrombotic thrombocytopenic purpura (TTP). 9 In TTP, Santoriello et al 10 Inagaki et al 11 Ardalan et al 12 Esmaili et al 13 Singh et al 14 Bae et al 15 Wu et al 17 Fraga-Rodriguez et al 18 Akyüz et al 3 Cupit-Link et al 19 Chiang et al 20 Thaunat et al 16 Davies microvascular aggregation commonly occurs in the brain; however, in HUS platelet-fibrin thrombus mostly affects the kidney. Furthermore, the normal level of ADAMTS 13 in HUS is useful in differentiating HUS from TTP.…”

“…2 Typical or diarrhea-positive HUS is associated with Shiga toxin-producing Escherichia coli and almost exclusively occurs in the childhood period. 3 Atypical hemolytic uremic syndrome (aHUS) is unrelated to Shiga toxins, accounts for around 5% to 10% of all cases, and has a poor prognosis. 4 Hemophagocytic lymphohistiocytosis (HLH) is a lifethreatening disorder of the mononuclear phagocytic system.…”

Eculizumab in a child with atypical haemolytic uraemic syndrome and haemophagocytic lymphohistiocytosis triggered by cytomegalovirus infection

“…Only one prior paediatric case of comorbid aHUS and HLH has been described—an 8-year-old boy presenting with low C3 which was managed with plasma exchange, fresh frozen plasma, haemodialysis, erythrocyte transfusion and low-dose oral steroids. 8 No information on ADAMTS13 levels or genetic/functional analysis of complement regulatory proteins was reported, and the follow-up was only 45 days. An earlier paper described an 18-year-old girl with TMA (kidney biopsy) and haemophagocytic syndrome that were probably secondary to infection, although no causal agent was identified.…”

Combined hemophagocytic syndrome and thrombotic microangiopathy due to mixed infection with influenza virus and pneumococcal pneumonia

“…Recently, we experienced a patient who developed hyperinflammatory lymphohistiocytosis (HLH)/hemophagocytic syndrome (HPS) and thrombotic microangiopathy (TMA) induced by community‐acquired pneumonia due to mixed infection with S pneumoniae and influenza virus. To our knowledge, only 3 cases of combined HLH/HPS and TMA have been reported, and there is only a single published report of TMA induced by pneumonia due to mixed infection with S pneumoniae and influenza virus …”