Natascha Ching, MD
Pediatric Infectious Disease
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Rodolfo Bégué, MD
Pediatric Infectious Disease
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
David Kurahara, MD
Pediatric Rheumatologist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Marian Melish, MD
Pediatric Infectious Disease
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Jennifer Di Rocco, MD
Pediatric Hospitalist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Kara Yamamoto, MD
Pediatric Rheumatologist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Jasmin Jensen, MD
Pediatric Hematologist-Oncologist
Kapiolani Medical Center for Women and Children, United States
Disclosure information not submitted.
Prashant Purohit, MD FAAP
Pediatric Intensivist
Kapiolani Medical Center for Women and Children, Hawaii, United States
Disclosure information not submitted.
Title: Multiorgan Failure and Hyperinflammation: A Rare Diagnosis of MIS-C by SARS-CoV-2 Spike Antibody
Case Report Body: A 15 years old obese male with diabetes mellitus presented with fever for over a month. He developed acute respiratory failure requiring mechanical ventilation, acute renal failure requiring CRRT, thrombocytopenia, and anemia requiring multiple transfusions, elevated D-dimer, direct hyperbilirubinemia, septic shock, and lactic acidosis. Cytokine panel showed elevated IL2R, IL4, Il10, IL13, IL6 and IL8; that along with ESR > 120, CRP peak of 228 mg/L, ferritin 11547 ng/ml were consistent with hyperinflammation. He had multiple negative blood, respiratory, urine and fungus cultures and metagenomic blood tests. Two bone marrow biopsies showed dysplastic and mini-megakaryocytes, increased non-clonal population of plasma cells, with normal cellularity and karyotype. The PET scan showed non-specific low intensity FDG uptake in multiple lymph nodes (LN), spleen and BM. CT abdomen showed hepatomegaly with diffuse fatty infiltration, mild splenomegaly, and reactive retroperitoneal LN. Echocardiogram and CT brain were unremarkable. He had multiple negative SARS CoV2 NAAT tests and nucleocapsid antibody tests.
Low-grade lymphoma, TTP, and HLH were considered possible diagnoses. His ADAMTS13 activity was moderately low (22% and repeat 29%, range > 61%). However, he had normal LDH/haptoglobin, with paucity of schistocytes on peripheral smear. Extensive autoimmune work-up, immune deficiency and acute leukemia panels were negative. Whole-exome sequencing showed SPINK-1 related hereditary pancreatitis, otherwise not significant.
SARS CoV2 Spike total antibody test was sent and showed initially 4.8 and later 323.6 (reference range < 0.8 U/mL). Since he was not immunized against SARS CoV2, this was diagnostic of MIS-C per CDC guidelines. He received steroids, IVIG followed by anakinra, and tocilizumab. There was a gradual but near-complete resolution of inflammatory markers, and clinically improving trajectory of his multiple organ failure.
Message: It remains unclear if he has underlying HLH which was precipitated by SARS CoV2, or MIS-C in the entirety. However, they both are rare presentations. The diagnostic challenges were more noteworthy with final establishment of diagnosis by newly available SARS Cov2 spike protein antibody testing. We hope to provide this insight with this rare case report.