Successful treatment with recombinant thrombomodulin for B-cell lymphoma-associated hemophagocytic syndrome complicated by disseminated intravascular coagulation

Masahiro Uni; Akihide Yoshimi; Hiroaki Maki; Daichi Maeda; Kumi Nakazaki; Fumihiko Nakamura; Masashi Fukayama; Mineo Kurokawa

Successful treatment with recombinant thrombomodulin for B-cell lymphoma-associated hemophagocytic syndrome complicated by disseminated intravascular coagulation

Int J Clin Exp Pathol. 2013 May 15;6(6):1190-4. Print 2013.

Authors

Masahiro Uni¹, Akihide Yoshimi, Hiroaki Maki, Daichi Maeda, Kumi Nakazaki, Fumihiko Nakamura, Masashi Fukayama, Mineo Kurokawa

Affiliation

¹ Department of Hematology & Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

PMID: 23696942
PMCID: PMC3657377

Abstract

We report here a 47-year-old male with the diagnosis of high-grade B-cell lymphoma and hemophagocytosis accompanying disseminated intravascular coagulation (DIC). Lymphoma-associated hemophagocytic syndrome (LAHS) is a life-threatening disorder, and LAHS secondary to B-cell lymphoma is relatively rare compared to that secondary to T- or NK/T-cell lymphoma in Western countries. T- or NK/T-cell LAHS is sometimes combined with DIC, which makes patients' outcomes even worse, but few reports of B-cell LAHS accompanying DIC has been published so far. We successfully treated a patient with this condition with recombinant thrombomodulin (rTM), a novel agent for DIC. We believe that rTM is a therapeutic option in cases with B-cell LAHS accompanying DIC.

Keywords: B-cell lymphoma; disseminated intravascular coagulation; lymphoma-associated hemophagocytic syndrome; recombinant thrombomodulin.

Publication types

Case Reports

MeSH terms

Antibodies, Monoclonal, Murine-Derived / administration & dosage
Antineoplastic Combined Chemotherapy Protocols / administration & dosage
Bone Marrow Examination
Cyclophosphamide / administration & dosage
Disseminated Intravascular Coagulation / diagnosis
Disseminated Intravascular Coagulation / drug therapy*
Disseminated Intravascular Coagulation / etiology
Doxorubicin / administration & dosage
Humans
Lymphohistiocytosis, Hemophagocytic / diagnosis
Lymphohistiocytosis, Hemophagocytic / drug therapy*
Lymphohistiocytosis, Hemophagocytic / etiology
Lymphoma, B-Cell / complications*
Lymphoma, B-Cell / drug therapy
Lymphoma, B-Cell / pathology
Male
Middle Aged
Neoplasm Grading
Paraneoplastic Syndromes / diagnosis
Paraneoplastic Syndromes / drug therapy*
Paraneoplastic Syndromes / etiology
Prednisone / administration & dosage
Recombinant Proteins / therapeutic use
Rituximab
Thrombomodulin / therapeutic use*
Treatment Outcome
Vincristine / administration & dosage

Substances

Antibodies, Monoclonal, Murine-Derived
R-CHOP protocol
Recombinant Proteins
Thrombomodulin
Rituximab
Vincristine
Doxorubicin
Cyclophosphamide
Prednisone