Fatal Hypogammaglobulinemia 3 Years after Rituximab in a Patient with Immune Thrombocytopenia: An Underlying Genetic Predisposition?

Table 1

Summary of the patient’s immunological profile.

Parameter	Normal range	At ITP diagnosis (2008)	6 months before RTX	Baseline	6 months post-RTX	Jul 2015	Nov 2015	May 2016
Blood hemoglobin, g/dL	12–16	14.8	12.1	13.7	14.3	14.5	13.6	11.9
Blood platelets, G/L	150–400	3	2	24	368	49	322	404
Blood leukocytes, /mm^3	4000–10,000	6,300	3,750	5,800	4,890	4,100	5,200	6,600
Blood neutrophils, /mm^3	2,500–7,500	4,570	2,670	4,950	3,590	120	3,100	5,430
Monocytes, /mm^3	200–1,000	370		190	270	600	370	400
Total blood gamma-globulins, g/L	7.8–16	8.4		8.9	ND	2.3	2.6	2.8
IgG	6.90–14	ND	ND	ND	ND	2.19	2.37	2.86
IgA	0.70–3.70	ND	ND	ND	ND	0.477	0.52	0.51
IgM	0.40–2.40	ND	ND	ND	ND	0.11	0	0.2
Total lymphocytes/mm^3		1,199	513	589	705	3,000	861	402
CD19^+ B cells, %	11–16	18.13	ND	16.75	0	0	0	0
CD19^+ B cells/mm^3	200–400	217	ND	99	0	0	0	0
CD3^+ T cells/mm^3	1,100–1,700	923	422	453	607	2,949	757	317
CD4^+T cells/mm^3	700–1,100	745	330	359	456	2,774	589	252
CD8^+ T cells/mm^3	500–900	138	69	74	131	132	151	54
CD4^+/CD8^+ ratio		5.39	4.78	4.85	3.5	21	12	4.6
CD3^+CD4^–CD8^–T cells/mm^3		37	21	18	17	43	40	7
γδ T Lymphocytes/mm^3		ND	ND	ND	6	ND	ND	3
CD4^+DR^+ T cells, %		4.03	7.88	4.5	3.7	59.1	ND	9.59
CD8^+DR^+ T cells, %		16.67	29	17.8	5.5	19.7	ND	12.96
CD3^–CD16^+CD56^+ NK/mm^3	200–400	ND	ND	18	7	27	ND	20

Baseline, 2012 just prior to first rituximab infusion. ND: not determined. NR: normal range, NK: natural killers.