Revealing the true face behind the mask of ALK-positive anaplastic large cell lymphoma (ALCL)

Hannah Eisfeld, Stefan Kircher, Andreas Rosenwald, Ioannis Anagnostopoulos, Mathias Werner, Nikolaus Gaßler, Gunter Wolf, Lukas Lehmkuhl, Ulf Teichgräber, Falk Gühne, Andreas Darr, Martin Freesmeyer (+6 others)
2020 Annals of Hematology  
Dear Editor, Anaplastic large cell lymphoma (ALCL) is the third most common peripheral T cell lymphoma [1] and is divided into anaplastic lymphoma receptor kinase (ALK)-positive and ALK-negative ALCL [2] . In 80% of ALK-positive ALCL, ALK is constitutively activated as a result of nucleophosmin (NPM)-ALK translocation. ALK belongs to the insulin receptor superfamily [3, 4] . Whereas the 5-year overall survival in ALK-positive ALCL is 70%, patients with ALK-negative ALCL have a 5-year survival
more » ... 32-50% [1, 5, 6]. On histology, ALCL shows large pleomorphic neoplastic cells with often horseshoe-shaped nuclei and strong expression of CD30, a cytokine receptor from the tumor necrosis factor receptor family [7, 8] . However, besides these characteristic findings, there is a broad range of morphological variations [8, 9] . We present an unusual case of a 38-year-old female patient with challenging diagnostic workup of an ALK-positive ALCL due to the variable histology and clinical course of this lymphoma. The patient initially complained of lower back pain and fever. FDG-positron emission tomography and computerized tomography (PET-CT) of the thorax and abdomen revealed multiple bone lesions (Fig. 1a, b) . The patient had an unremarkable medical and family history and no occurrence of B-symptoms. Clinical examination showed no abnormalities. All other examinations including bronchoscopy, gynecologic assessment, gastroscopy, colonoscopy, and abdominal ultrasound did not reveal any abnormalities. The laboratory results were within normal limits except elevated C-reactive protein (CRP) levels with 56.9 mg/l (normal range < 7.5 mg/l). A CT-guided core needle biopsy of an osteolytic lesion of the left iliac bone was performed. The histopathologic results showed an infectious or inflammatory process. No malignancies could be diagnosed; however, CD30-positive cells were found at low frequency, which could not be further evaluated due to the scarcity of the available tissue (Fig. 1c) . A second bone biopsy of this lesion revealed metaplastic woven bone with chronic inflammatory infiltration, consistent with chronic recurrent multifocal osteomyelitis (CRMO) (Fig. 1f ). Bisphosphonates and corticosteroid
doi:10.1007/s00277-020-04247-4 pmid:32895754 fatcat:s5xhlrfz7jg33p4r3nymey76ti