Immunological and clinical characteristics of severe thrombocytopenia in neonates with Kasabach-Merritt phenomenon.
This study aimed to characterize the immune profile of neonates with Kasabach-Merritt phenomenon (KMP) and to identify clinical features linked to severe thrombocytopenia in these patients.
This multicenter retrospective study included neonates diagnosed with KMP based on established criteria. The peripheral blood lymphocyte subsets, complement levels, and humoral immunity were compared between KMP neonates and controls. KMP neonates were further divided into severe and non-severe groups based on whether the platelet count was <20 × 109/L. Clinical data and treatment outcomes were compared between the two groups, and multivariate analysis was performed to identify independent factors associated with severe thrombocytopenia.
A total of 32 KMP neonates were included (13 in the severe group and 19 in the non-severe group). Compared with controls, KMP neonates had reduced CD3+CD4+ and CD3-CD19+ proportions and lower IgG levels (all P < 0.05). The most common type of KHE lesion in KMP neonates was superficial + mixed (24/32, 75.00%), with a median maximum diameter of 68.50 (52.50-87.25) mm and a platelet count of 41.50 (17.25-48.75) × 109/L. Of the KMP neonates, 25 achieved complete remission (78.1%), 4 achieved partial remission (12.5%), and 3 had no remission (9.3%). Compared to the non-severe group, the severe KMP group had larger diameters. The severe group also had lower CD3+CD4+ proportions, and significantly higher levels of lymphocytes percentage (L%), PCT, CKMB, and ferritin, along with prolonged APTT (P < 0.05). No significant difference in prognosis was observed between the two groups (P > 0.05). Multivariate regression analysis identified prolonged APTT and elevated ferritin as independently associated with severe thrombocytopenia in KMP.
Neonates with KMP showed immunological abnormalities, including decreases in helper T cells and B cells. Prolonged APTT and elevated ferritin were identified as independent risk factors for severe thrombocytopenia in KMP.
This multicenter retrospective study included neonates diagnosed with KMP based on established criteria. The peripheral blood lymphocyte subsets, complement levels, and humoral immunity were compared between KMP neonates and controls. KMP neonates were further divided into severe and non-severe groups based on whether the platelet count was <20 × 109/L. Clinical data and treatment outcomes were compared between the two groups, and multivariate analysis was performed to identify independent factors associated with severe thrombocytopenia.
A total of 32 KMP neonates were included (13 in the severe group and 19 in the non-severe group). Compared with controls, KMP neonates had reduced CD3+CD4+ and CD3-CD19+ proportions and lower IgG levels (all P < 0.05). The most common type of KHE lesion in KMP neonates was superficial + mixed (24/32, 75.00%), with a median maximum diameter of 68.50 (52.50-87.25) mm and a platelet count of 41.50 (17.25-48.75) × 109/L. Of the KMP neonates, 25 achieved complete remission (78.1%), 4 achieved partial remission (12.5%), and 3 had no remission (9.3%). Compared to the non-severe group, the severe KMP group had larger diameters. The severe group also had lower CD3+CD4+ proportions, and significantly higher levels of lymphocytes percentage (L%), PCT, CKMB, and ferritin, along with prolonged APTT (P < 0.05). No significant difference in prognosis was observed between the two groups (P > 0.05). Multivariate regression analysis identified prolonged APTT and elevated ferritin as independently associated with severe thrombocytopenia in KMP.
Neonates with KMP showed immunological abnormalities, including decreases in helper T cells and B cells. Prolonged APTT and elevated ferritin were identified as independent risk factors for severe thrombocytopenia in KMP.
Authors
Li Li, Li Li, Huang Huang, Gao Gao, Li Li, Hu Hu, Feng Feng, Zhu Zhu, Geng Geng
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