Overlap Connective Tissue Disease Presenting with Secondary Myelofibrosis: A Rare Case Report

Abstract

Systemic sclerosis (SSc) and rheumatoid arthritis (RA) overlap syndromes are uncommon autoimmune diseases that share clinical and serological characteristics. In these patients, hematological problems such secondary myelofibrosis are quite rare. We present a 30-year-old man from Jamalpur, Bangladesh, who has had progressive skin tightness for 1.5 months and polyarticular pain for 3 months. Blackish leg ulcers, Raynaud’s phenomenon, microstomia, sclerodactyly, and symmetrical small joint pain with prolonged morning stiffness were among his symptoms. Rheumatoid factor, anti-cyclic citrullinated peptide, and anti-Scl-70 antibodies were shown to be positive in laboratory tests. Features of overlap between RA and SSc were confirmed by imaging and skin biopsy procedures. He developed acute pancytopenia while receiving corticosteroids, vasodilators, and methotrexate. Pre-fibrotic myelofibrosis was identified as an uncommon hematological consequence of connective tissue overlap syndrome by peripheral blood film and bone marrow biopsy. When treating patients with connective tissue overlap syndromes, clinicians should keep a high index of suspicion for hematologic complications, such as myelofibrosis. Improving outcomes needs multidisciplinary management and early diagnosis.

Bangladesh J Medicine 2026; Vol. 37, No. 2(1): pp. 234