Session: Lymphocytes, Lymphocyte Activation and Immunodeficiency, including HIV and Other Infections: Poster III
Poster Board III-687
Post Splenectomy Clinical Course:
Cytopenias: Post-splenectomy, 19 patients (56%) developed or relapsed with multilineage cytopenias of grade 2 or higher including anemia (29%), neutropenia (26%), and thrombocytopenia (44%). Most of these patients experienced recurrence of multiple episodes of cytopenias involving more than one lineage post-splenectomy requiring further treatment interventions. To control refractory post-splenectomy cytopenias, 26% (5/19) of them needed long-term steroid sparing measure using MMF.
Sepsis: Ten patients developed sepsis post-splenectomy. Of the 32 patients with information on prophylactic antibiotic use post-splenectomy to prevent pneumococcal sepsis, 22 (69%) received antibiotics (Penicillin 14, others 8). However, 8 (36%) of the 22 patients that received prophylactic antibiotics developed sepsis. Sepsis occurred 8 months to 36 years post-splenectomy. Two patients with sepsis were critically ill requiring hospitalization in intensive care and one died at home before receiving medical care. Among these 10 patients, 9 developed pneumococcal sepsis ranging from 1-6 episodes per patient (median 2). Two (6% of the total cohort of 34) patients died due to sepsis post-splenectomy. Relationship between prophylactic antibiotics use and risk of sepsis is difficult to interpret because in this observational study those most at risk for sepsis could have been the ones more likely to receive antibiotics. Information related to vaccine administration and pneumococcal titer responses were not available at the time of infection. Of interest, three patients belonging to one family, included here, developed pneumococcal sepsis post-splenectomy.
Lymphoma: There were 8 patients (24%) with B cell lymphoma in this cohort of 34 patients, one of whom developed lymphoma pre-splenectomy. Two asplenic adult patients with lymphoma developed pulmonary hypertension as adults. Two patients with lymphoma died due to non-infectious causes.
Overall ALPS-FAS patients have a high prevalence of recurrent cytopenias (56%) as well as sepsis (29%) following splenectomy. The morbidity and mortality associated with splenectomy, as well as the persistence of cytopenias, raise important questions as to the timing and efficacy of splenectomy in this rare disorder of immune dysfunction leading to multilineage cytopenias due to autoimmune destruction and splenic sequestration.
* Reference:Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome: report from the 2009 NIH International Workshop. Oliveira JB, Bleesing JJ, Dianzani U, Fleisher TA, Jaffe ES, Lenardo MJ, Rieux-Laucat F, Siegel RM, Su HC, Teachey DT, Rao VK.
Blood. 2010 Jun 10. PMID: 20538792.
Disclosures: No relevant conflicts of interest to declare.
See more of: Oral and Poster Abstracts
*signifies non-member of ASH