Pneumocystis jirovecii-associated immune reconstitution inflammatory syndrome-like phenomenon in a child with leukaemia: a case report and literature review

BMC Pediatr. 2022 Jul 12;22(1):410. doi: 10.1186/s12887-022-03441-9.

Abstract

Background: Immune reconstitution inflammatory syndrome (IRIS) refers to the phenomenon of intense immune responses against pathogens in patients with AIDS undergoing antiretroviral therapy to reconstitute immune function, resulting in functional impairment of multiple organs. Non-AIDS immunosuppressed hosts may also develop similar manifestations to IRIS during immune recovery.

Case presentation: An 8-year-old girl presented with acute lymphoblastic leukaemia was admitted for scheduled chemotherapy treatment. During chemotherapy, she experienced pancytopenia and Pneumocystis jirovecii pneumonia, which was diagnosed based on the abnormal shadows observed on chest computed tomography, the elevation of serum β-D-glucan, and the positive mNGS results of Pneumocystis jirovecii in both sputum and blood. After treatment with Granulocyte Colony-Stimulating Factor, sulfamethoxazole, and caspofungin, aggravation of lung lesions was discovered and severe interstitial lung disease developed in a short period along with a rapidly increasing leukocyte count. Intravenous methylprednisolone pulse therapy was given, but lung function did not improve, and she finally died after the withdrawal of medical care.

Conclusions: For patients with acute lymphocytic leukaemia infected with Pneumocystis jirovecii, the rapid aggravation of pulmonary lesions in the process of blood recovery and immune reconstitution should raise vigilance against the possibility of IRIS-like reactions. The use of granulocyte stimulating factors may aggravate the inflammatory response in the lungs. The timing, dosage, and duration of treatment of glucocorticoids and the impact of high-dose methylprednisolone pulse therapy on the prognosis of patients should be explored in further research.

Keywords: Acute lymphocytic leukaemia; Immune reconstitution inflammatory syndrome-like phenomenon; Pneumocystis Jirovecii pneumonia.

Publication types

  • Case Reports
  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Female
  • Humans
  • Immune Reconstitution Inflammatory Syndrome* / diagnosis
  • Immune Reconstitution Inflammatory Syndrome* / etiology
  • Leukemia*
  • Methylprednisolone
  • Pneumocystis carinii*
  • Pneumonia, Pneumocystis* / complications
  • Pneumonia, Pneumocystis* / diagnosis
  • Pneumonia, Pneumocystis* / drug therapy

Substances

  • Methylprednisolone