AUTOINFLAMMATORY DISEASES IN HEMATOLOGY FROM DIAGNOSIS TO TREATMENT

AUTOINFLAMMATORY_DISEASES_IN_HEMATOLOGY_FROM_DIAGNOSIS_TO_TREATMENTkapak

IHD with MGCS

Fadime Nurcan Alhan

Alanya Training and Research Hospital, Department of Hematology, Antalya, Türkiye

Alhan FN. IHD with MGCS. In: Kurt Yüksel M, editor. Autoinflammatory Diseases in Hematology from Diagnosis to Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.111-121.

ABSTRACT

Monoclonal gammopathies arise from the clonal proliferation of plasma cells or B-lymphocytes. The presence of monoclonal proteins without identified identifiable symptomatic diseases, such as plasma cell disorders and lymphomas, is classified as “monoclonal gammopathy of undetermined significance (MGUS).” Epidemiologically, MGUS is the most frequently diagnosed monoclonal gammopathy.

Uncontrolled plasma cell or B-lymphocyte proliferation can result in excessive monoclonal immunoglobulin or immunoglobulin fragment production. These molecules may accumulate in vital organs or exert biological activity, potentially leading to tissue damage. The histological features, clinical manifestations, prognosis, and treatments vary depending on the location and type of damage. Clinical conditions with nephrological, dermatological, rheumatological, neurological, and ophthalmological manifestations linked to monoclonal gammopathy are categorized as monoclonal gammopathies of clinical significance (MGCS). Initially identified in renal pathology as monoclonal gammopathy of renal significance (MGRS), MGCS now encompasses neurological (MGNS), cutaneous (MGSS), and ocular (MGOS) manifestations.

Unlike MGUS, MGCS necessitates treatment. However, due to the rarity and complexity of these disorders, standardized treatment protocols are lacking. Early suppression of the causative clone and monoclonal protein may prevent irreversible organ damage and associated morbidity.

Keywords: Kidney; Monoclonal gammopathy; Neuropathy; Skin

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