Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients.

  title={Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients.},
  author={Sigbj{\o}rn Berentsen and Elling Ulvestad and Bj{\o}rn Tore Gjertsen and Henrik Hjorth-Hansen and Ruth Langholm and H{\aa}var Knutsen and Waleed Ghanima and Fuad Victor Shammas and Geir Erland Tj{\o}nnfjord},
  volume={103 8},
Conventional therapies for primary chronic cold agglutinin disease (CAD) are ineffective, but remissions after treatment with the anti-CD20 antibody rituximab have been described in a small, prospective trial and in some case reports. In this study we report on 37 courses of rituximab administered prospectively to 27 patients. Fourteen of 27 patients responded to their first course of rituximab, and 6 of 10 responded to re-treatment. In both groups combined, responses were achieved after 20 of… 

Tables from this paper

Rituximab in chronic cold agglutinin disease: a prospective study of 20 patients

Previous findings of a favourable effect of rituximab in patients with CAD are confirmed, however, few patients will obtain CR and, in most patients, the effect will be transient.

High response rate and durable remissions following fludarabine and rituximab combination therapy for chronic cold agglutinin disease.

Fludarabine and rituximab combination therapy is very efficient in patients with CAD, and benefits should be carefully weighed against risks in very old and comorbid patients.

Bendamustine plus rituximab for chronic cold agglutinin disease: results of a Nordic prospective multicenter trial.

Bendamustine-rituximab combination therapy is highly efficient, sufficiently safe, and may be considered in first line for patients with CAD requiring therapy.

Rituximab-containing therapy for cold agglutinin disease: a retrospective study of 16 patients

A rituximab-based therapy in accordance with individual patient characteristics may be a reasonable choice for CAD patients.

Rituximab is an effective and safe therapeutic alternative in adults with refractory and severe autoimmune hemolytic anemia

Rituximab induced clinical responses in multitreated severe refractory both warm and cold AIHA patients with little toxicity, and consequently, this therapy should be considered as an early therapeutic option in this setting.

Rituximab is an effective and safe treatment of relapse in elderly patients with resistant warm AIHA

Rituximab is an effective treatment for elderly patients with refractory warm AIHA and any pretreatment characteristics predictive of response to ritUXimab are not identified.

Long-term observation of patients with anti-neutrophil cytoplasmic antibody-associated vasculitis treated with rituximab.

Rituximab is an effective and well-tolerated treatment for patients with ANCA-associated vasculitis and should be strongly considered in severely affected patients who do not respond to standard therapy or in those in whom cytotoxic therapy bears a high risk of morbidity.

Beneficial Effect of Rituximab in Combination with Oral Cyclophosphamide in Primary Chronic Cold Agglutinin Disease

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Low-dose rituximab in adult patients with idiopathic autoimmune hemolytic anemia: clinical efficacy and biologic studies.

The clinical response was correlated with amelioration biologic markers such as cytokine production (IFN-γ, IL-12, TNF-α, and IL-17), suggesting that low-dose rituximab exerts an immunomodulating activity.

How I manage cold agglutinin disease

  • S. Berentsen
  • Medicine, Biology
    British journal of haematology
  • 2011
Primary chronic cold agglutinin disease is a clonal lymphoproliferative disorder accounting for 13–15% of autoimmune haemolytic anaemias, and fludarabine‐rituximab combination therapy is very effective, resulting in 75% response rate, complete remissions in about 20%, and more than 66 months estimated response duration.



Favourable response to therapy with the anti‐CD20 monoclonal antibody rituximab in primary chronic cold agglutinin disease

A small, open, uncontrolled, prospective study to evaluate the effect of therapy with the monoclonal anti‐CD20 antibody rituximab in patients with chronic cold agglutinin disease, with encouraging results.

Rituximab in autoimmune cytopenias: for which patients?

The anti-CD20 monoclonal antibody rituximab represents a new tool for treating B-cell malignancies, especially low-grade lymphomas, and has been shown to be helpful in some patients with refractory autoimmune cytopenias, and the papers in this issue of Haematologica extend knowledge on these new, emerging indications.

Rituximab chimeric anti-CD20 monoclonal antibody therapy for relapsed indolent lymphoma: half of patients respond to a four-dose treatment program.

The response rate of 48% with IDEC-C2B8 is comparable to results with single-agent cytotoxic chemotherapy, and further investigation of this agent is warranted, including its use in conjunction with standard chemotherapy.

Beneficial effects of rituximab on primary cold agglutinin disease refractory to conventional therapy

Rituximab played a significant role in the treatment of refractory CAD associated with LPL, and the serum level of IgM decreased in association with the disappearance of lymphoma cells and clonal rearrangement of the Ig heavy chains in the bone marrow after treatment with ritUXimab.

No response to cladribine in five patients with chronic cold agglutinin disease

A small, open, uncontrolled, prospective study to evaluate the possible effect of cladribine therapy in patients with chronic cold agglutinin disease and the mechanism of action of these drugs is probably not phase dependent.

[Rituximab in cold agglutinin disease].

Rituximab is an alternative treatment of cold agglutinin disease and with a good tolerance, the treatment allowed a remission in all the cases (4 partial, 1 complete).

Rituximab therapy for indolent non‐Hodgkin's Iymphoma

Rituximab is therefore an excellent treatment option both as first‐line and as salvage therapy for patients with indolent NHL.

Clinical immunology of chronic cold agglutinin disease

The results indicate that some patients with CAD have a relative deficit of complement in their serum and that an increase of complement production occurs during an acute phase reaction which enhances haemolysis.

IDEC-C2B8: results of a phase I multiple-dose trial in patients with relapsed non-Hodgkin's lymphoma.

IDEC-C2B8 chimeric anti-CD20 mAb therapy is well tolerated and has clinical activity in patients with relapsed B-cell lymphoma and the 375-mg/m2 dose has been selected for a phase II trial.

Rituximab in a case of cold agglutinin disease

The dual testing rate for both lupus anticoagulant (LA) and solid-phase type assays must be used for the detection of antiphospholipid antibodies (aPLs) in certain patients is concluded to be poor.