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800 Autoimmune Cytopenia Associated with Indolent B-Cell Clones: A Large Multicentric Cohort Study

Program: Oral and Poster Abstracts
Type: Oral
Session: 311. Disorders of Platelet Number or Function: Clinical and Epidemiological: Thrombocytopenia in Special Populations
Hematology Disease Topics & Pathways:
Bleeding and Clotting, autoimmune disorders, Research, autoimmune hemolytic anemia, Lymphomas, platelet disorders, Clinical Research, Diseases, Immune Disorders, indolent lymphoma, Therapies, real-world evidence, Lymphoid Malignancies
Monday, December 11, 2023: 3:00 PM

Yoann Zadro1*, Amélie Lusque2*, Heloise Rey1*, Julie Graveleau3*, Fanny Lemarie4*, Lenaig Le Clech5*, Corentin Orvain6*, Paul Albert Domnariu7*, Clement Gourguechon8*, Etienne Crickx, MD, PhD9*, Kamel Laribi10*, Delphine Gobert11*, Leonce Adjoumani12*, Emilie Berthoux13*, Alban Deroux14*, Marie Durel15*, Mickael Ebbo16*, Jonathan Farhi17*, Sebastien Humbert18*, Vincent Jachiet19*, Pierre Cougoul1*, Jeremie Dion1*, Brice Castel20*, Clement Begey21*, Jean-Baptiste Rieu1*, Samuel Deshayes22*, Mathilde Devaux23*, Isabelle Durieu24*, Martin Gauthier25*, Jean-Francois Viallard, MD PhD26*, Nihal Martis27*, Jérôme Paillassa28*, Guillaume Denis29*, Sondess Hadj Khelifa30*, Christian Lavigne17*, Francois Lifermann, MD31*, Guillaume Moulis32*, Arsene Mekinian19*, Loic Ysebaert1*, Marc Michel9*, Bertrand Godeau9* and Thibault Comont1*

1Toulouse University Hospital, Toulouse, France
2Institut Claudius Regaud - Institut Universitaire Du Cancer De Toulouse-Oncopole, Toulouse, FRA
3Saint Nazaire Hospital, Saint Nazaire, France
4CH Cornouaille, Quimper, France
5CH Lorient, Quimper, FRA
6Angers University Hospital, Anges, France
7Bicetre Hospital APHP, Paris, France
8Amiens University Hospital, Amiens, France
9Internal medicine department, Henri Mondor Hospital, APHP, Créteil, France
10Hematology Department, Le Mans Hospital, Le Mans, France
11Saint Antoine Hospital APHP, Paris, FRA
12Avignon Hospital, Avignon, France
13Saint Joseph - Saint Luc Hospital, Lyon, France
14Service De MéDecine Interne, CHU Grenoble-Alpes, La Tronche, FRA
15Robert Schuman Hospital, Metz, France
16CHU Marseille, Marseille, FRA
17Angers University Hospital, Angers, France
18Besancon University Hospital, Besancon, FRA
19Saint Antoine Hospital AP-HP, Paris, France
20Centre Hospitalier de Bigorre, Tarbes, France
21Besancon University Hospital, Besancon, France
22Caen University Hospital, Caen, France
23CHI Poissy St Germain en Laye, Poissy, France
24Service de Médecine Interne, Université De Lyon- Hospices Civils De Lyon, Pierre Benite, France
25Cahors Hospital, Cahors, France
26Service Médecine Interne, Haut-Leveque Hospital, Pessac, France
27CHU De Nice, Nice, FRA
28Centre Hospitalier Universitaire d' Angers, Angers, France
29Rochefort Hospital, Rochefort, FRA
30Auch Hospital, Auch, France
31Dax Hospital, DAX CEDEX, FRA
32Toulouse University hospital, Toulouse, France


Autoimmune cytopenias (AIC) are a group of rare diseases sharing immune-induced accelerated destruction and/or impaired production of blood cells. AIC can be associated with overt B-cell lymphoid neoplasia. In that case, the management is based on the treatment of the hematological malignancy. However, AIC can be associated with indolent B-cell clones (IBC). In that case, the treatment of the AIC is not consensual. We aimed to describe the treatments used in France for IBC-associated AIC, and to compare their effectiveness, safety, and impact on IBC progression.


We conducted a multicenter, retrospective observational study including adults treated between 2000 and 2022 for a definite AIC (immune thrombocytopenia, ITP; autoimmune hemolytic anemia, AIHA; or Evans syndrome, ES) associated with an IBC, namely: chronic lymphocytic leukemia (CLL), marginal zone lymphoma (MZL), lymphoplasmacytic lymphoma, monoclonal B lymphocytosis, and mantle cell lymphoma. Patients who required a specific treatment of the underlying IBC for one or several of the following reason(s) were excluded: extensive marrow infiltration leading to impaired megacaryopoiesis and/or erythropoiesis, massive organ infiltration and/or compressive lymphadenopathies, lymphocytosis doubling within 6 months (with a lymphocytes count >30 x 109/L), doubling of lymphadenopathy size within 2 months, hyperviscosity, and any other IBC-related condition requiring treatment (amyloidosis, neuropathy...).

Patients were classified according first-line treatment for AIC: group 1 for those treated without chemotherapy (corticosteroids, intravenous immunoglobulin, rituximab...); group 2 for those treated with chemotherapy (cyclophosphamide, ibrutinib...). Only the first-line treatment was assessed. Response to treatment was defined according to standard international criteria. An infection was defined as serious if graded >2, according to the Common Terminology Criteria for Adverse Events. The progression was defined as the need to urge lymphoma-specific therapy. Subgroup analyses were performed by type of AIC (ITP, warm/mixed AIHA, cold agglutinin disease, and ES). Overall survival was estimated using the Kaplan-Meier method and comparison was performed using Log-rank test.


In total, 187 patients were included throughout 30 French university and general hospitals using the French reference center network for immune cytopenias in adults. The median age at diagnosis of AIC was 75 years (range 43 to 97 years) with a male/female ratio of 1.47 (Table 1). We observed 105 (56.1%) patients with AIHA, 80 (42.8%) with CLL and 44 (23.5%) with MZL. In most cases (58.8%), the time elapsed between IBC and AIC diagnosis was £6 months.

We included 132 (72.2%) patients in group 1. Among them, 75 (55.6%) patients were treated with only corticosteroids +/- intravenous immunoglobulin, 52 (38.5%) with rituximab, 2 with splenectomy, and 1 with dapsone. We included 52 (27.8%) patients in group 2. Among them, 46 (88.5%) patients were treated as first-line with a rituximab-cyclophosphamide-dexamethasone combined regimen, 2 with rituximab-bendamustine, 2 with chloraminophen, 1 with fludarabine-cyclophosphamide-dexamethasone, and 1 with ibrutinib.

Both overall response and relapse rates were similar between groups: 86.6% versus 86.5% and 45.5% versus 41.5%, respectively. We did not find any difference in subgroup analyses by type of AIC.

Serious infections were observed in 17.6% patients: 17.8% in group 1, 17.3% in group 2.

With a median of follow-up of 39.8 months (95% IC 30.0 to 51.3), the overall survival did not differ between group 1 (84.0% at 4 years, 95% IC 73.7 to 90.5) and group 2 (81.8% at 4 years, 95% IC 66.1 to 90.7), nor the progression rate of IBC was similar in both groups (9.6% and 7.7%, respectively).


We report the largest cohort of AIC associated with IBC. So far, very few patients with lymphomas such as MZL have been described in this situation. We observed that treatments were heterogeneous. Whatever the strategy used (treating AIC only or also using chemotherapy for the IBC), the effectiveness and safety profiles of first-line treatments were not different. Additional analyses are ongoing to better clarify the impact of rituximab alone versus rituximab and chemotherapy, and IBC’ characteristics on the overall progression-free survival.

Disclosures: Crickx: Novartis: Honoraria; Sanofi: Honoraria; UCB: Honoraria. Viallard: EUSAPHARMA: Consultancy. Paillassa: Incyte: Honoraria. Moulis: Sanofi: Honoraria, Research Funding; Amgen: Honoraria, Research Funding; Grifols: Honoraria, Research Funding; Argenx: Honoraria; Novartis: Honoraria, Research Funding. Michel: Sanofi: Consultancy; UCB: Honoraria; Alexion: Consultancy; Sobi: Consultancy; argenx: Honoraria; Novartis: Consultancy. Godeau: Novartis: Honoraria; Amgen: Honoraria; Sobi: Honoraria; Grifols: Honoraria. Comont: Amgen: Honoraria; Novartis: Honoraria, Speakers Bureau; BMS: Consultancy, Speakers Bureau.

*signifies non-member of ASH