Disorders induced by deposits of monoclonal immunoglobulin IgM and free light chain in Waldenström’s macroglobulinaemia – case report and review of literature

Czech version

Authors: P. Flodr ¹; Z. Adam ²; M. Navratilova ¹; D. Holub ³; P. Džubák ³; L. Křen ⁴; J. Dobrovolný ⁵; P. Malíková ⁵; O. Pollaková ⁶; M. Šenkyřík ⁷; L. Zdražilová Dubská ⁸; Z. Řehák ⁹; R. Koukalová ⁹; M. Krejčí ¹; L. Pour ¹; Z. Král ¹; I. Svobodová ¹⁰
Authors‘ workplace: Ústav klinické a molekulární patologie, LF UP a FN Olomouc ¹; Interní hematologická a onkologická klinika LF MU a FN Brno ²; Ústav molekulární a translační medicíny, LF UP, Olomouc ³; Ústav patologie, LF MU a FN Brno ⁴; Hematologicko-transfuzní oddělení, Institut Klinické a Experimentální Medicíny (IKEM) Praha ⁵; Hematologické oddělení, Nemocnice Nové Město na Moravě ⁶; Interní gastroenterologická klinika LF MU a FN Brno ⁷; Katedra laboratorních metod LF MU a Ústav laboratorní medicíny FN Brno ⁸; Oddělení nukleární medicíny, Masarykův onkologický ústav Brno ⁹; I. ústav patologie, FN u sv. Anny v Brně ¹⁰
Published in: Transfuze Hematol. dnes,30, 2024, No. 2, p. 99-111.
Category: Review/Educational Papers
doi: https://doi.org/10.48095/cctahd2024prolekare.cz8

Overview

SUMMARY: Waldenström’s macroglobulinaemia (WM) is a low-grade B-cell lymphoproliferative disorder characterised by an immunoglobulin IgM monoclonal gammopathy and bone marrow infiltration by lymphoplasmacytic lymphoma. Clinical features may be related to the overall disease burden, such as anaemia, thrombocytopenia, and constitutional inflammatory symptoms, or may be directly attributable to the IgM paraprotein. The concentration of monoclonal IgM can vary widely in WM. There is no direct relationship between the concentration of monoclonal immunoglobulin IgM and bone marrow infiltration. The spectrum of monoclonal immunoglobulin IgM-related disorders is large because of the diversity of involved organs and pathogenic mechanisms. Lesions commonly result from the deposition of all or part of the M-IgM as aggregates, amorphous, crystalline, microtubular, or fibrillar forms. Other mechanisms include autoantibody activity against a tissue antigen, formation of immune complexes, and complement activation. In addition, even a small B-cell clone may absorb biologically active molecules or induce cytokine secretion. In our case report, we describe a female patient with monoclonal IgM and lambda liver deposition and we discuss the frequency and variety of disorders caused by deposition of monoclonal IgM and free light chain.

Keywords:

hepatomegaly – Amyloidosis – Waldenström’s macroglobulinaemia – Monoclonal Immunoglobulin IgM Deposition Disease – macroglobulinosis

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GRANTOVÁ PODPORA

Publikace byla vytvořena na podporu těchto aktivit: MZ ČR – RVO (FNBr, 65269705), a Institucionální aktivity MOÚ: MZ ČR – RVO (MOÚ, 00209805).

ČESTNÉ PROHLÁŠENÍ

Autoři prohlašují, že jejich autorství na článku je nepřivádí do žádného konfliktu zájmů a nikdo z autorů nemá jakoukoliv ekonomickou vazbu na nezdravotnické ekonomické subjekty.

PODÍL AUTORŮ NA PŘÍPRAVĚ RUKOPISU

PF – hodnotil histologii a podílel se na textu z úhlu pohledu své odbornosti

ZA – sepsal text, ošetřoval pacientku na ambulanci a koordinovat spolupráci všech spoluautorů

MN, DH, PD – podíleli se na analýze vzorku jater v Amyloidovém centru v Olomouci. Dále se podíleli na tvorbě textu z úhlu pohledu jejich odbornosti

LK – provedl první hodnocení biopsie jater v Brně a spolupráce s Amyloidovým centrem v Olomouci a podílel se na textu z úhlu pohledu své odbornosti

JD, PM – stanovili diagnózu Waldenströmovy makroglobulinémie za pobytu pacientky v IKEM Praha, spolupracovali na textu z jejich zorného úhlu pohledu na popsaný případ

OP – vyšetřovala, ošetřovala a ošetřuje pacientku v místě bydliště a doplnila text o informace o počátcích onemocnění

MŠ – provedl biopsie jater a podílel se na tvorbě textu z úhlu pohledu gastroenterologa

ZŘ, RK – provedli FDG-PET/CT zobrazení a spolupracovali na textu z úhlu pohledu nukleární medicíny

LZD – vyšetřila monoklonálního imunoglobulin a spolupracovala na textu z úhlu pohledu biochemika specializovaného na analýzu monoklonálních imunoglobulinů

IS – hodnotila endomykoardiální biopsii

LP, MK, ZK – se podíleli na péči o pacientku a přípravě textu

Doručeno do redakce dne: 6. 2. 2024.

Přijato po recenzi dne: 29. 2. 2024.

MUDr. Patrik Flodr

Ústav klinické a molekulární patologie

LF UP a FN Olomouc

Hněvotínská 3

775 15 Olomouc

e-mail: patrik.flodr@fnol.cz

prof. MUDr. Zdeněk Adam, CSc.

Interní hematologická a onkologická klinika