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Gamma Heavy-Chain Disease Accompanied with Follicular

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Gamma Heavy-Chain Disease Accompanied with Follicular Case report Gamma heavy-chain disease accompanied with follicular lymphoma: a case report Paula San-José1, Vicente Aguadero*1, Granada Perea1,2, Meritxell Estrada1,2, Eugenio Berlanga1 1Clinical Laboratory, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain 2Hematology Department, Parc Taulí Hospital Universitari, Institut d’Investigació i Innovació Parc Taulí I3PT, Universitat Autònoma de Barcelona, Spain *Corresponding author: [email protected] Abstract Heavy chain diseases (HCD) are B-cell lymphoprolipherative disorders characterized by the production of monoclonal heavy chains without asso- ciated light chains. Some cases of gamma-HCD (γ-HCD) are concurrent with other lymphoid neoplasm. The monoclonal component is not always detectable by serum electrophoresis, and often an immunofixation procedure is necessary to detect this component. Prognosis is variable, and no established guidelines for follow-up are available. We describe a case of a challenging diagnosis of γ-HCD due to the absence of clinical signs frequ- ently reported in the disease (anaemia and palatal oedema among others). Haematological malignancy was the first suspicion but bone marrow examination was negative. In addition, the presence of an autoimmune bicytopenia and a Klinefelter syndrome complicated the clinical context of the patient. A thoracoabdominal computed tomography reported many small adenopathies whose pathological and immunohystochemical study revealed a follicular lymphoma. Shortly after, serum inmunofixation secondary to an abnormal electrophoretic pattern revealed a gamma para- protein without light chains. Eventually, γ-HCD in association with follicular lymphoma was the final diagnosis. This is the first case reporting this association. Key words: paraproteins; electrophoresis; lymphoma; heavy chain disease Received: August 25, 2017 Accepted: December 08, 2017 Introduction Heavy chain disease (HCD) is a rare B-cell lym- scribed in the literature (4). Although γ-HCD has phoproliferative disorder characterized by the been reported to occur equally in men and wom- production of monoclonal, abnormally truncated, en, a slight predominance in women has recently immunoglobulin heavy-chain proteins (alpha, been noted (5). The median age of diagnosis is 65 gamma, and/or mu) without associated light years old (3). chains (1,2). Heavy chain disease can be thought of Classic presentation includes generalized lymphad- as a variant of non-Hodgkin’s lymphoma. Clinical enopathies and splenomegaly (2). The most distinc- manifestations depend on the chain isotype in- tive symptom is palatal oedema resulting from en- volved and range from asymptomatic to aggres- largement of nodes in Waldeyer’s ring, sometimes sive lymphoma. Prognosis is variable, and no leading to respiratory compromise (3,5). standardized effective treatment programs are Gamma-HCD has no specific histopathologic pat- available (2,3). tern. The most frequent histopathological finding Gamma-HCD (γ-HCD), also known as Franklin’s dis- is a pleomorphic malignant lymphoplasmacytic ease, was reported for the first time in 1964. Since proliferation in bone-marrow and lymph nodes. then, approximately 130 cases have been de- Aspirate and biopsy specimens may show an in- https://doi.org/10.11613/BM.2018.010802 Biochem Med (Zagreb) 2018;28(1):010802 ©Copyright by Croatian Society of Medical Biochemistry and Laboratory Medicine. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creative- commons.org/licenses/by/4.0/) which permits users to read, download, copy, distribute, print, search, or link to the full texts of these articles in any medium or format and to remix, transform and build upon 1 the material, provided the original work is properly cited and any changes properly indicated . San-José P. et al. Gamma heavy-chain disease: A case report crease in plasma cells, lymphocytes, and/or plas- Cobas 8000 (Roche Diagnostics, Mannheim, Ger- macytoid lymphocytes (similar to the bone-mar- many). Laboratory findings on admission are sum- row findings in Waldenström macroglobulinemia) marized in Table 1. Considering CBC, the results (3,6). Nevertheless, in some cases γ-HCD are con- obtained indicated neutropenia and thrombocy- current with other lymphoid neoplasms, including topenia whereas haemoglobin was normal. A pe- Hodgkin and non-Hodgkin lymphomas, which ripheral blood smear did not show dysplastic fea- usually precede the serologic diagnosis of γ-HCD tures for any haematopoietic lineage and had no (2,7,8). circulating blasts or erythroblasts. Biochemical in- One third of these cases are associated with auto- dicators of liver function were slightly increased, immune diseases such as rheumatoid arthritis, au- while the β-2 microglobulin concentration was toimmune haemolytic anaemia, or idiopathic above the normal values. thrombocytopenic purpura (5,6). Some authors di- In suspicion of haematological malignancy, a bone vide γ-HCD into three broad categories based on marrow aspirate and biopsy were requested. They the underlying pathologic process and its distribu- showed normocellular bone marrow with the tion: 1) those with disseminated proliferative dis- presence of three haematopoietic lineages with ease (55 - 60%); 2) those with localized prolifera- no remarkable dysplastic features and no matura- tive disease (25%); and 3) those with no apparent tion arrest in granulocytic lineage. There was no proliferative disease (10 - 15%) (3,6). evidence of infiltration of either tumour cells or In this study, we present a case of a 50-year male atypical lymph cells. An incidental finding such as with gamma heavy chain disease accompanied a chromosomal abnormality (47, XXY) was detect- with follicular lymphoma, a subtype of non-Hodg- ed by cytogenetic study. He was diagnosed with kin lymphoma according to WHO classification (9), Klinefelter syndrome. and explain the challenge of its diagnosis. Since the bone marrow study was negative, immu- nological examination including antinuclear anti- Case report bodies, anti-mitochondrial, anti-gastric parietal cells, and anti-smooth-muscle was performed by A 50-year-old obese, male smoker with excessive indirect immunofluorescent tests using the com- alcohol consumption and a medical history of mercially available kit NOVA Lite Rat (Inova Diag- chronic obstructive pulmonary disease was re- nostics, San Diego, California). All tests were nega- ferred for a haematology consultation due to a bi- tive. Furthermore, immunoglobulin (Ig) A, G, and cytopenia detected during a routine examination M serum concentrations were investigated on Im- in October 2016. No masses or visceromegaly were mage 800 analyser (Beckman Coulter, Brea, Cali- found during first physical exam. Following physi- fornia). Results are presented in Table 1. cal exam, haematology and biochemical analyses Additionally, different serological tests were exam- were performed. Samples for complete blood ined: hepatitis and HIV on Cobas e411 (Roche diag- count (CBC) were collected from the cubital veins nostics, Mannheim, Germany); mumps, Epstein- into blood collection Vacuette® tubes with K3EDTA Barr virus and cytomegalovirus on Liaison (Diasor- (Greiner Bio-one, Kremsmunster, Austria) and were in, Saluggia, Italy). All these tests were negative measured on Sysmex XN 9000 (Sysmex, Kobe, Ja- (Table 1). pan) whereas peripheral blood smear was per- formed by microscopy. Samples for biochemical Following serological examination, serum electro- analysis were collected into blood collection Vacu- phoretic pattern was evaluated on Capillarys 2 (Se- ette® tubes with serum clot activator and gel bia, Lisse, France). At that moment it was normal as (Greiner Bio-one, Kremsmunster, Austria) for as- shown in Figure 1A. Finally, presence of antibodies partate-aminotranspherase (AST), lactate dehy- by immunofluorescence was investigated and re- drogenase (LD), beta(β)-2 microglobulin and C-re- sults revealed presence of IgG and IgM anti-neu- active protein (CRP), and were measured on the trophil antibodies, attached to cell surface (direct Biochem Med (Zagreb) 2018;28(1):010802 https://doi.org/10.11613/BM.2018.010802 2 San-José P. et al. Gamma heavy-chain disease: A case report TABLE 1. Laboratory findings on admission and during follow up Admission Follow up Test group Parameter Reference intervals (October 2016) (February 2017) WBC (x109/L) 1.3 1.33 4-11 Neutrophils (x109/L) 0.1 0.48 2.5-7.5 Lymphocytes (x109/L) 0.52 0.31 1-4.5 CBC Monocytes (x109/L) 0.59 0.43 0.2–1.0 Haemoglobin (g/L) 143 166 130-175 Platelets (x109/L) 54 93 130-400 AST (U/L) 47 44 0-38 LD (U/L) 308 251 135-225 Chemistry β-2 MG (µg/mL) 3.7 3.9 0.8-2.2 CRP (mg/L) 44 - 0-5 IgG (g/L) 7.64 10.60 7.00–16.00 Immunology IgA (g/L) 0.90 1.37 0.7–4.0 IgM (g/L) 3.62 4.03 0.4-2.3 HBSAG Negative - Negative HBCAC Negative - Negative HCAC Negative - Negative HIVAC Negative - Negative EBIGM Negative - Negative Serology EBIGG Positive - Negative CMVIGM Negative - Negative CMVIGG Positive - Negative MUMPSIGG Negative - Negative MUMPSIGM Negative - Negative Anti-neutrophil antibodies Positive* - Negative Autoimmunity Anti-platelet antibodies Positive† - Negative *Presence of IgG and IgM anti-neutrophil antibodies attached to cell surface (direct test) and free in serum (indirect test). †IgG anti-platelet antibodies (cut-off > 30%). CBC - complete blood count. WBC - white blood cell count. AST – aspartate-amino- transferase.
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