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Table of Contents
Vol. 126, No. 2, 2011
Issue release date: August 2011
Section title: Case Report
Acta Haematol 2011;126:103–109
(DOI:10.1159/000327251)

Paroxysmal Nocturnal Hemoglobinuria after Autologous Stem Cell Transplantation: Extinction of the Clone during Treatment with Eculizumab – Pathophysiological Implications of a Unique Clinical Case

Pulini S.a · Marando L.c · Natale A.a · Pascariello C.c, d · Catinella V.b · Del Vecchio L.c, d · Risitano A.M.c · Fioritoni G.a
aDepartment of Hematology, and bDepartment of Transfusion Medicine, Flow Cytometry Section, ‘Spirito Santo’ Hospital, Pescara, cDepartment of Biochemistry and Medical Biotechnologies, Federico II University, and dCEINGE, Advanced Biotechnologies, Naples, Italy

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Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: November 09, 2010
Accepted: February 24, 2011
Published online: May 19, 2011
Issue release date: August 2011

Number of Print Pages: 7
Number of Figures: 3
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

The clinical and biological spectrum of paroxysmal nocturnal hemoglobinuria (PNH) is variable, ranging from classical hemolytic forms to PNH associated with aplastic anemia or other bone marrow (BM) failure syndromes. We report a previously undescribed case of PNH occurring after autologous stem cell transplantation (ASCT) in a patient affected by relapsing non-Hodgkin’s lymphoma. The intensive chemotherapy and the ASCT resulted in a contraction of the effective hematopoietic stem cell (HSC) pool and a derangement of the immune system. The delayed engraftment and the BM hypoplasia represented a favorable environment for the expansion of the pathological clone. This case is paradigmatic even for the unexpected trend of the PNH clone during treatment with the terminal complement inhibitor eculizumab; in fact, the clone reduced until undergoing unexpected extinction, i.e. the recovery of normal hematopoiesis. Eculizumab seems not to play a direct role in HSC kinetics; the clinical remission probably occurred because the environmental conditions that led to the expansion of the PNH clone were transient and disappeared.

© 2011 S. Karger AG, Basel


Article / Publication Details

First-Page Preview
Abstract of Case Report

Received: November 09, 2010
Accepted: February 24, 2011
Published online: May 19, 2011
Issue release date: August 2011

Number of Print Pages: 7
Number of Figures: 3
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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