Diffuse Large B-Cell Lymphoma with Cryagglutininemia: A Case Report

Case Report

Ann Hematol Onco. 2023; 10(4): 1429.

Diffuse Large B-Cell Lymphoma with Cryagglutininemia: A Case Report

Jiang N#; Liu CY#; Fu R*

Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan Street, Heping District, Tianjin, China

*Corresponding author: Fu R Department of Hematology, Tianjin Medical University General Hospital, 154 Anshan Street, Tianjin 300052, People’s Republic of China. Email: furong8369@tmu.edu.cn

#These author have contributed equally to this article.

Received: June 05, 2023 Accepted: June 30, 2023 Published: July 07, 2023

Abstract

Diffuse Large B-Cell Lymphoma (DLBCL) is the most common non-Hodgkin’s lymphoma in the world. Cryoagglutininemia is an autoimmune disease caused by cold inducing factors and characterized by chronic hemolytic anemia and microcirculation embolism, and it mainly involves IgM antibody and may be secondary to lymphoma, and other diseases. We reported 1 case of DLBCL with cryoagglutininemia. The patient was 39 years old, female, diagnosed as DLBCL stage IVB with aaIPI score = 2 at moderate/high risk. She presented the onset symptoms of generalized skin petechiae and ecchymosis. The physical examination showed a 2×2cm lump at the right 3rd rib on the midclavicular line. The patient was diagnosed as cryoagglutininemia. After admission to our hospital, she was diagnosed as DLBCL by transcostal lump puncture biopsy and then treated with the chemotherapy of Zanubrutinib + CHOPE regimen for 4 cycles. Thereafter, PET-CT reexamination indicated that Deauville score was 2, the treatment was effective, and the disease was relieved.

Keywords: Diffuse large B-cell lymphoma (DLBCL); Cryoagglutininemia; Treatment; Bruton tyrosine kinase (BTK); Zanubrutinib; Case report

Introduction

Diffuse large B-cell lymphoma (DLBCL) is the most common invasive malignant lymphoma and accounts for 30%-40% of all cases in different regions [1]. We present a 39-year-old female patient with DLBCL and cryoagglutininemia the patient was treated with the chemotherapy of Zanubrutinib + CHOPE regimen for 4 cycles. Thereafter, PET-CT reexamination indicated that the disease was relieved. The treatment process is reported below.

Results

Investigations

There was a 39-years-old female patient. Over half a month ago, the patient repeatedly presented bright red petechiae and ecchymosis on legs and popliteal fossae, as well as urticarioid plaques on the abdomen and axillary fossae without reasons, accompanied by fever. The physical examination showed a 1×1cm lump on the left oral mucosa nearby the gingiva with palpable pain and a 2×2cm lump at the right 3rd rib on the midclavicular line with palpable pain. The patient sought for medical care successively in the First Affiliated Hospital of Zhengzhou University and Peking Union Medical College Hospital.

Diagnosis

The relevant examinations were completed. The results showed: serum protein electrophoresis: M-protein 1.9%, cryoglobulin (+); cold agglutinin test: 4o 1:512 (+), 37o rewarming (-); Coombs (+); IgM Kappa type; M-protein (-). Cryoagglutininemia was diagnosed. On October 12, 2021, the patient was firstly hospitalized in our department for treatment. After admission, PET-CT examination showed multiple bone destructions in the scanning range and abnormally increased metabolism, thus malignancy was considered. The bone marrow puncture indicated a myelogram of granulocyte, erythrocyte and megakaryocyte hyperplasia and an increased proportion of erythrocytes. The bone marrow biopsy showed: active myeloproliferation, a slightly decreased proportion of granulocytes and erythrocytes, visible cells in various phases dominated by mature cells (CD34 (+, occasional), CD117 (-), Lysozyme (+, sparse), MPO (+, occasional)), an increased number of B lymphocytes in diffuse, sparse and multi-focal distribution (CD20, CD19, Bcl-2: +, multi-focal; CD10: +, occasional; CD21, CD23, CD5, Bcl-6, SOX-11, CycinD1: -; CD3, CD138: +, sparse), normal morphology of megakaryocytes (CD61 (+)); MM phenotype: the abnormal plasmocytes accounted for 0.085% of karyocytes, expressing CD38, CD138, CD56 and kappa, and partially expressing CD45, of which 0.018% plasmocytes did not express CD56; lymphocyte phenotype: the abnormal cell clusters accounted for 19.15%, expressing CD19, CD20 and Kappa, and partially expressing CD5, thus they were considered as monoclonal B lymphocytes; chromosome: 45, XX [11]; LPL/WM gene test: MYD88 (primary variation), BCL2 (tertiary variation); DLBCL hotspot gene screening: MYD88 (primary variation); bone marrow lymphoma FISH test: IGH rearrangement (+); in-situ lymphoma cell leukemia FISH test: negative. On October 22, 2021, a CT-guided right rib lump puncture was performed. The findings showed: B cell lymphoma, probable DLBCL, non-GCB-derived; immunohistochemistry: positive CD20, CD19, Mum-1 and CyclinD1, sparsely weak positive Bcl-6, negative CD10, CD3, CD5, SOX-11, TdT, CD56, CD38, CD138, CD235a, CD34, CD117 and CD61, and partially positive Kappa and Lambda in tumor cells. Combining the clinical manifestations and examination results, DLBCL stage IVB with aaIPI score =2 at moderate/high risk accompanied by cryoagglutininemia were definitely diagnosed.

Citation: Jiang N, Liu CY, Fu R. Diffuse Large B-Cell Lymphoma with Cryagglutininemia: A Case Report. Ann Hematol Onco. 2023; 10(4): 1429.