Pancytopenia as an early indicator for Stevens-Johnson syndrome complicated with hemophagocytic lymphohistiocytosis: a case report

Abstract

BackgroundStevens-Johnson syndrome (SJS) is a severe skin and mucosal bullous disease. When complicated with Hemophagocytic lymphohistiocytosis (HLH), the condition is especially life-threatening.Case presentationHere we report the case of a 4-year-old boy suffering from SJS with extensive erythema multiforme and bulla. Despite active intervention and supportive care, the boy experienced increased skin lesions and a higher fever. Meanwhile, decreases in white blood cell count and hemoglobin were observed. Hyperferritinemia, increased soluble CD25 level, decreased NK cell activity and hemophagocytosis in the boy’s bone marrow confirmed the diagnosis of HLH. After high-dose intravenous immunoglobulin and methylprednisone pulse therapy, the boy was discharged in good condition.ConclusionSimultaneous occurrence of HLH and SJS is very uncommon and the condition is life-threatening. Pancytopenia can be a precocious indicator and enables to start a prompt diagnosis and treatment.

Association between serum ferritin and the severity of drug eruptions

“…Our study revealed that serum ferritin level has a predictive value for the severity of drug eruptions in the early stage. It was significantly higher in the SCADRs group compared with the non‐SCADRs group, a result that is consistent with some case reports and studies showing the association between hyperferritinemia and drug eruptions in SJS and DRESS . The association between ferritin and some variables, which may reflect the severity of drug eruptions and physical condition of patients, was observed.…”

Toxic epidermal necrolysis and hemophagocytic lymphohistiocytosis: a case report and literature review

“…Our patient's favorable outcome is consistent with other pediatric case reports . One patient relapsed , but was effectively managed, demonstrating the need for close surveillance.…”

“…Our case describes a boy with a confirmed viral and bacterial prodrome, followed by a presumptive immune drug response. A two-hit hypothesis has support in three other cases [12,13,16], whereby nonspecific viral upper respiratory tract symptoms temporally overlapped with medications commonly implicated with TEN. Two other TEN cases [11,17] reported no mucosal involvement and isolated EBV from the skin lesions, suggesting a single viral entity.…”

“…Membranous desquamation prior to HLH diagnosis has been documented in case reports [11][12][13]. This may be a spurious observation, or herald a pivotable aspect of disease progression.…”

Secondary haemophagocytic lymphohistiocytosis associated with metronidazole

“…The causative agents in most reported cases are antibiotics or anticonvulsants including trimethoprim–sulfamethoxazole, vancomycin, phenytoin, phenobarbital and lamotrigine 2 3. HLH is mostly associated with severe hypersensitivity reactions such as Stevens-Johnson syndrome, toxic epidermal necrolysis4 or drug reactions with eosinophilia and systemic symptoms 2. These reactions form part of the type 4 delayed-hypersensitivity reaction, which involves activation of drug-specific T cells.…”