Hemophagocytic lymphohistiocytosis complicating invasive pneumococcal disease: a pediatric case report

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is an infrequent but life-threatening disease due to excessive immune activation. Secondary HLH can be triggered by infections, autoimmune diseases, and malignant diseases. Streptococcus pneumoniae is a pathogenic bacterium responsible for invasive pneumococcal disease (IPD) such as meningitis and bacteremia. Although the pneumococcal conjugate vaccine (PCV) has led to reductions in IPD incidence, cases of IPD caused by serotypes not included in PCV are increasing. There are few reports of secondary HLH caused by IPD in previously healthy children. We herein report a rare case of a previously healthy boy with secondary HLH complicating IPD of serotype 23A, which is not included in the pneumococcal 13-valent conjugate vaccine (PCV-13). Case presentation: An 11-month-old boy who had received three doses of PCV-13 was hospitalized with prolonged fever, bilateral otitis media, neutropenia and elevated C-reactive protein (CRP) levels. Blood culture on admission revealed S. pneumoniae, leading to a diagnosis of IPD. HLH was diagnosed based on a prolonged fever, neutropenia, anemia, hepatosplenomegaly, hemophagocytosis in the bone marrow, and elevated serum levels of triglycerides, ferritin, and soluble interleukin-2 receptor. He received broad-spectrum antibiotics and intravenous immunoglobulins for IPD and high-dose steroid pulse therapy and cyclosporine A for HLH; thereafter, his fever resolved, and laboratory findings improved. The serotype of the isolated S. pneumoniae was 23A, which is not included in PCV-13. Conclusions: It is important to consider secondary HLH as a complication of IPD cases with febrile cytopenia or hepatosplenomegaly, and appropriate treatment for HLH should be started without delay.

Etiology, Treatment and Outcome of Children Diagnosed with Secondary Hemophagocytic Lymphohistiocytosis in A Tertiary Hospital

“…Thereafter, clinical status and blood tests started to improve. 17 A chest radiograph finding of interstitial pneumonia was also frequently seen in patients with EBV-HLH. 3 Many patients were admitted at the intensive care unit because of complications associated with a delay in diagnosis.…”

The first case of Streptococcus intermedius brain abscess with hemophagocytic histiocytosis

“…In Japan, the incidence of childhood HLH caused by bacterial infection accounts for about 6% of all IAHS cases [ 3 ], including Streptococcus pneumonia , Mycobacterium tuberculosis , Leptospira, Brucella, and group B hemolytic streptococcus, Streptococcus suis , Haemophilus influenzae , Klebsiella pneumonia , Staphylococcus aureus , Mycobacterium bovis , Salmonella typhimurium , Mycoplasma pneumonia , Q fever, Jungle typhus, etc. [ 4 – 9 ]. There is no report of S. intermedius brain abscess-induced HLH mentioned in literature before.…”

Secondary hemophagocytic lymphohistiocytosis in children (Review)

“…Ehrlichia chaffeensis, the agent of human monocytic ehrlichiosis, has been reported from the US (118)(119)(120)(121)(122). A case of S. pneumoniae 23A, serotype that is not included in the pneumococcal 13-valent conjugated vaccine (PCV-13) has been reported from Japan (123).…”