Autoimmune haemolytic anaemia associated with mantle cell lymphoma

Abstract

Autoimmune haemolytic anaemia (AIHA) is a well-recognised complication of lymphoproliferative disorders, and has been reported in association with all B and T cell non-Hodgkin lymphoma subtypes with the exception of mantle cell lymphoma (MCL). We describe herein a case of MCL diagnosed in an initially asymptomatic 66-year-old woman who developed transfusion-dependent AIHA 6 months later coincident with lymphoma progression. The AIHA failed to respond satisfactorily to conventional treatment (high-dose oral prednisolone) but rapidly resolved following commencement of non-rituximab-containing combination chemotherapy in parallel with complete remission of the lymphoma. This is the first of such cases to be described in the literature and confirms that the immune environment of MCL can predispose to AIHA in the same way as in other lymphoma subtypes. Despite this being an infrequent occurrence, clinicians should be aware that AIHA is a potential complication of MCL and may be more successfully controlled by treating the underlying lymphoma rather than relying on conventional anti-haemolytic strategies such as steroids.

Autoimmune haemolytic anaemia in mantle cell lymphoma : an insidious complication associated with leukemic disease

“…This patient may have developed immune complex glomerulonephritis, but, in the absence of any organ biopsy, this could not be definitively proven. A quite similar observation, which was luckily clinically less relevant, was reported by Eve et al [4]. Overall, immunochemotherapy seemed the most active therapy for treating AIHA.…”

“…Patient 5 developed AIHA at first relapse, and was the only blastoid variant of the series. Patient 4 developed AIHA shortly before lymphoma progression, and failed to respond to high-dose oral prednisolone, but rapidly improved following combination chemotherapy in parallel with lymphoma remission, resembling the case described in the literature by Eve et al [4]. Patient 5 displayed an aggressive clinical course despite intensive immunochemotherapy, being now in second early relapse.…”

“…The clinical and biological characteristics of our patients suggest that this complication Alive and free of disease (6) Died of rapid disease progression (4) Alive and free of disease (5) Alive but with ongoing disease progression (12) MCL, mantle cell lymphoma; AIHA, developing autoimmune haemolytic anemia; IFT, immunophenotype obtained by flow cytometry and/or immunohistochemistry, all cases being CD19+ and CD20+; BM, bone marrow; PB, peripheral blood; IGHV, immunoglobulin heavy chain variable region genes; NA, not assessed; DAT, direct antiglobulin test; NE, not evaluable because administered together with chemotherapy; R, rituximab; CVP, cyclophosphamide, vincristine, prednisone; PBSC, peripheral blood stem cell transplant; BAC, bendamustine, cytarabine; MOF, multiorgan failure; BR, bendamustine and rituximab; SOX11 was + when expressed by immunohistochemistry at the nuclear level. Ann Arbor stage A or B: the absence of constitutional (B-type) symptoms is denoted by adding an "A" to the stage.…”

“…Development of AIHA secondary to MCL appears an extremely infrequent occurrence, with only one case described in the literature [4]. The occurrence of immune thrombocytopenia is also rare in MCL [2,5], suggesting that immune-mediated complications overall are much less common in MCL than other subtypes.…”

A case of primary ovarian lymphoma with autoimmune hemolytic anemia achieving complete response with Rituximab-based combination chemotherapy

“…[11] This occurrence of AIHA with various lymphomas is also known but extremely rare with DLBCL. [612]…”