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Epidemiology

Hematological manifestations of Good syndrome

Leukemia volume 35, pages 1817–1819 (2021)Cite this article

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We read with great interest the letter by Gurnari et al. reporting the coexistence of pure red cell aplasia (PRCA) and large granular lymphocytic leukemia with Good syndrome, an adult-onset acquired immunodeficiency [1]. Autoimmunity is a recognized feature of Good syndrome, and PRCA is the most frequent immune-mediated hematological manifestation. To complement their findings of this rare disease, we report the hematological manifestations of Good syndrome based on a comprehensive systematic literature review. Differences of clinical features, management, and outcomes between patients with and without hematological manifestations were explored.

Of 162 patients included in the current study, the median age at diagnosis was 58 years (interquartile range [IQR] 51-67), and 51.0% were male (Table 1). Infections were recorded in 150 patients (92.6%), and WHO type AB (50.0%) was the most frequent histological subtype of thymoma. Among 83 patients with concurrent autoimmunity, PRCA is the most common hematological manifestation (26/83, 31.3%), and other findings include pure white cell aplasia (6/83, 7.2%), aplastic anemia (4/83, 4.8%), and immune thrombocytopenia (2/83, 2.4%) (Supplementary Table 1). In total, 37 patients had immune-mediated hematological manifestations, in which one patient had PRCA and immune thrombocytopenia diagnosed consecutively along the course of Good syndrome. No difference was noted in clinical features and basic immunological findings, except for a significantly higher frequency of recurrent pulmonary infections in those with hematological manifestations (64.9% vs. 44.0%, p = 0.026). In terms of management, more patients with hematological manifestations received additional immunosuppressive therapy (59.5% vs. 16.0%, p < 0.001), and other treatments were similar among the two groups.

Table 1 Comparison of Good syndrome patients with and without immune-mediated hematological manifestations.
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Fig. 1: Hematological manifestations of Good syndrome.

References

  1. Gurnari C, Durrani J, Pagliuca S, Kishtagari A, Awada H, Kerr CM, et al. Novel invariant features of Good syndrome. Leukemia. 2021. https://doi.org/10.1038/s41375-020-01114-z.

  2. Kelesidis T, Yang O. Good’s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135:347–63.

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  1. Department of Internal Medicine, Morsani College of Medicine, University of South Florida, Tampa, FL, USA

    Chen Wang

  2. Department of Medicine, The Warren Alpert Medical School of Brown University, Providence, RI, USA

    Yiyun Shi

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  1. Chen Wang
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Correspondence to Chen Wang.

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Wang, C., Shi, Y. Hematological manifestations of Good syndrome. Leukemia 35, 1817–1819 (2021). https://doi.org/10.1038/s41375-021-01255-9

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