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Case Reports
. 2021 Jun;35(6):1792-1796.
doi: 10.1038/s41375-020-01114-z. Epub 2021 Jan 7.

Novel invariant features of Good syndrome

Carmelo Gurnari #  1   2 , Jibran Durrani #  1 , Simona Pagliuca  1 , Ashwin Kishtagari  1 , Hassan Awada  1 , Cassandra M Kerr  1 , Vera Adema  1 , Sunisa Kongkiatkamon  1 , Alan E Lichtin  1   3 , Bhumika J Patel  1   3 , Valeria Visconte  1 , Mikkael A Sekeres  1   3 , James R Cook  4 , Jaroslaw P Maciejewski  5   6
Affiliations
Case Reports

Novel invariant features of Good syndrome

Carmelo Gurnari et al. Leukemia. 2021 Jun.
No abstract available

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Conflict of interest statement

Compliance with ethical standards

Conflict of interest The authors declare that they have no conflict of interest.

Figures

Fig. 1
Fig. 1. Overlapping relationships between Good syndrome, large granular lymphocytic leukemia, and pure red cell aplasia.
A Venn diagram representation and circle plot of the overlapping relationship of Good syndrome with LGL and PRCA. The Venn diagram circles indicate overlap (not proportional in size to the prevalence of these diseases). B Bar chart illustrates the frequencies of each clinical condition across the different cohorts (numbers above each of the bars are the total numbers of patients in each category). C Demographic and hematological characteristics of our case series of patients. UPN 1 is the original patient who was identified to have the tetrad of diseases. T-cell receptor gamma rearrangement was noted in all patients. UPN 1, 2, and 3 noted to have very low levels of absolute reticulocyte counts consistent with the diagnosis of pure red cell aplasia. D Five-year overall survival of patients with Good syndrome (66%, 47.9–90.5; median follow-up time of 64 months (range, 0.4–227). E Idealized heat map generated to demonstrate combinatorial configuration of clinical features, e.g, immunoglobulin levels, PRCA, and LGL. Normal ranges were considered according to the followings: IgG 717–1411 mg/dL, IgA 78–391 mg/dL, IgM 53–334 mg/dL. ANC absolute neutrophils count, GS good syndrome, Hb hemoglobin, Hypogamma hypogammaglobulinemia, Ig immunoglobulin, LGL Large granular lymphocytic leukemia, M male, retic reticulocytes, PRCA pure red cell aplasia, TCR T-cell receptor rearrangement, WBC white blood cell.
See this image and copyright information in PMC

Comment in

References

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