Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenström’s macroglobulinemia with bortezomib

Mayuko Izumi, Hiroko Tsunemine, Yasuhiro Suzuki, Akihiro Tomita, Toshiko Kusumoto, Taiichi Kodaka, Kiminari Itoh, Takayuki Takahashi

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenström’s macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9 g/dL, serum IgM concentration of 1904 mg/dL, and a titer of cold hemagglutinin of over ×8192. Serum immunoelectrophoresis demonstrated monoclonal protein of the IgM-κ type. A bone marrow aspirate showed many lymphoplasmacytic cells, which were positive for CD19, CD20, CD38, and cytoplasmic μ and κ light chains. A diagnosis of WM-associated cold hemagglutinemia was made. Because of red blood cell transfusion-dependency, we treated him with intravenous fludarabine, oral melphalan–prednisolone, cyclophosphamide, and melphalan, and two courses of R-CHOP in sequence with a marked decrease of serum IgM (928 mg). We then started weekly bortezomib plus dexamethasone (BD) therapy, as he was still transfusion-dependent. Soon after the initiation of BD, he achieved transfusion independence, with a further decrease in serum levels of IgM and marked improvement of anemia. Interestingly, his marrow abnormal lymphocytes were later found not to carry the MYD88 L265P mutation. The successful treatment with bortezomib for WM lacking this mutation is discussed.

Original languageEnglish
Pages (from-to)238-243
Number of pages6
JournalInternational Journal of Hematology
Volume102
Issue number2
DOIs
Publication statusPublished - 11-08-2015

Fingerprint

Waldenstrom Macroglobulinemia
Immunoglobulin M
Mutation
Serum
Dexamethasone
Bone Marrow
Erythrocyte Transfusion
Immunoelectrophoresis
Melphalan
Hemagglutinins
Therapeutics
Hemolysis
Cyclophosphamide
Anemia
Hemoglobins
Lymphocytes
Light
Bortezomib
Proteins

All Science Journal Classification (ASJC) codes

  • Hematology

Cite this

Izumi, Mayuko ; Tsunemine, Hiroko ; Suzuki, Yasuhiro ; Tomita, Akihiro ; Kusumoto, Toshiko ; Kodaka, Taiichi ; Itoh, Kiminari ; Takahashi, Takayuki. / Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenström’s macroglobulinemia with bortezomib. In: International Journal of Hematology. 2015 ; Vol. 102, No. 2. pp. 238-243.
@article{0a70b99ed23c434d9007f87252ae1c29,
title = "Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenstr{\"o}m’s macroglobulinemia with bortezomib",
abstract = "We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenstr{\"o}m’s macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9 g/dL, serum IgM concentration of 1904 mg/dL, and a titer of cold hemagglutinin of over ×8192. Serum immunoelectrophoresis demonstrated monoclonal protein of the IgM-κ type. A bone marrow aspirate showed many lymphoplasmacytic cells, which were positive for CD19, CD20, CD38, and cytoplasmic μ and κ light chains. A diagnosis of WM-associated cold hemagglutinemia was made. Because of red blood cell transfusion-dependency, we treated him with intravenous fludarabine, oral melphalan–prednisolone, cyclophosphamide, and melphalan, and two courses of R-CHOP in sequence with a marked decrease of serum IgM (928 mg). We then started weekly bortezomib plus dexamethasone (BD) therapy, as he was still transfusion-dependent. Soon after the initiation of BD, he achieved transfusion independence, with a further decrease in serum levels of IgM and marked improvement of anemia. Interestingly, his marrow abnormal lymphocytes were later found not to carry the MYD88 L265P mutation. The successful treatment with bortezomib for WM lacking this mutation is discussed.",
author = "Mayuko Izumi and Hiroko Tsunemine and Yasuhiro Suzuki and Akihiro Tomita and Toshiko Kusumoto and Taiichi Kodaka and Kiminari Itoh and Takayuki Takahashi",
year = "2015",
month = "8",
day = "11",
doi = "10.1007/s12185-015-1775-3",
language = "English",
volume = "102",
pages = "238--243",
journal = "International Journal of Hematology",
issn = "0925-5710",
publisher = "Springer Japan",
number = "2",

}

Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenström’s macroglobulinemia with bortezomib. / Izumi, Mayuko; Tsunemine, Hiroko; Suzuki, Yasuhiro; Tomita, Akihiro; Kusumoto, Toshiko; Kodaka, Taiichi; Itoh, Kiminari; Takahashi, Takayuki.

In: International Journal of Hematology, Vol. 102, No. 2, 11.08.2015, p. 238-243.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Successful treatment of refractory cold hemagglutinemia in MYD88 L265P mutation-negative Waldenström’s macroglobulinemia with bortezomib

AU - Izumi, Mayuko

AU - Tsunemine, Hiroko

AU - Suzuki, Yasuhiro

AU - Tomita, Akihiro

AU - Kusumoto, Toshiko

AU - Kodaka, Taiichi

AU - Itoh, Kiminari

AU - Takahashi, Takayuki

PY - 2015/8/11

Y1 - 2015/8/11

N2 - We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenström’s macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9 g/dL, serum IgM concentration of 1904 mg/dL, and a titer of cold hemagglutinin of over ×8192. Serum immunoelectrophoresis demonstrated monoclonal protein of the IgM-κ type. A bone marrow aspirate showed many lymphoplasmacytic cells, which were positive for CD19, CD20, CD38, and cytoplasmic μ and κ light chains. A diagnosis of WM-associated cold hemagglutinemia was made. Because of red blood cell transfusion-dependency, we treated him with intravenous fludarabine, oral melphalan–prednisolone, cyclophosphamide, and melphalan, and two courses of R-CHOP in sequence with a marked decrease of serum IgM (928 mg). We then started weekly bortezomib plus dexamethasone (BD) therapy, as he was still transfusion-dependent. Soon after the initiation of BD, he achieved transfusion independence, with a further decrease in serum levels of IgM and marked improvement of anemia. Interestingly, his marrow abnormal lymphocytes were later found not to carry the MYD88 L265P mutation. The successful treatment with bortezomib for WM lacking this mutation is discussed.

AB - We report here the successful treatment of cold agglutinin-associated refractory hemolysis with bortezomib in a patient with Waldenström’s macroglobulinemia (WM). A 78-year-old man was referred to our hospital with cold hemagglutinemia of unknown cause. Laboratory examination revealed a hemoglobin concentration of 6.9 g/dL, serum IgM concentration of 1904 mg/dL, and a titer of cold hemagglutinin of over ×8192. Serum immunoelectrophoresis demonstrated monoclonal protein of the IgM-κ type. A bone marrow aspirate showed many lymphoplasmacytic cells, which were positive for CD19, CD20, CD38, and cytoplasmic μ and κ light chains. A diagnosis of WM-associated cold hemagglutinemia was made. Because of red blood cell transfusion-dependency, we treated him with intravenous fludarabine, oral melphalan–prednisolone, cyclophosphamide, and melphalan, and two courses of R-CHOP in sequence with a marked decrease of serum IgM (928 mg). We then started weekly bortezomib plus dexamethasone (BD) therapy, as he was still transfusion-dependent. Soon after the initiation of BD, he achieved transfusion independence, with a further decrease in serum levels of IgM and marked improvement of anemia. Interestingly, his marrow abnormal lymphocytes were later found not to carry the MYD88 L265P mutation. The successful treatment with bortezomib for WM lacking this mutation is discussed.

UR - http://www.scopus.com/inward/record.url?scp=84938949214&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84938949214&partnerID=8YFLogxK

U2 - 10.1007/s12185-015-1775-3

DO - 10.1007/s12185-015-1775-3

M3 - Article

C2 - 25794560

AN - SCOPUS:84938949214

VL - 102

SP - 238

EP - 243

JO - International Journal of Hematology

JF - International Journal of Hematology

SN - 0925-5710

IS - 2

ER -