Transformation to plasmablastic lymphoma in CLL upon ibrutinib treatment

Abstract

Chronic lymphatic leukaemia (CLL) is the most common leukaemia in the Western world. Ibrutinib, a tyrosine kinase inhibitor, is the treatment of choice on relapse or p53-dysfunction. Richter's transformation to diffuse large B cell lymphoma is most often seen. However, transformation to other aggressive lymphomas as plasmablastic lymphoma (PBL) does occur. PBL is an extremely aggressive lymphoma and is usually treated using a CHOP-like regimen (cyclophosphamide, doxorubicin, vincristine and prednisone/dexamethasone), but with poor outcome. The only curative treatment is allogeneic stem cell transplant (ASCT).We report on a case of CLL treated with ibrutinib that underwent transformation to PBL. Due to high expression of CD138, we added daratumumab to the chemotherapy with a good, but transitory response. The case did not make it to an ASCT. Targeting CD138 by daratumumab may be added to chemoimmune therapy for PBL.

Keywords: chemotherapy; haematology (incl blood transfusion).

Figure 1

(A) H&E-stained section (× 60) from bone marrow showing large cells with plasmablastic differentiation with abundant cytoplasm, eccentrically placed nuclei and prominent nucleoli. (B) H&E-stained section (× 60) from lymph node showing the same large atypical lymphoid cells with plasmablastic features as seen in the bone marrow. (C) Immunohistochemical study showed the atypical lymphoid cells were positive for CD138. Example from bone marrow (× 40) shown here.

Figure 2

(A, B) CT scan at admission disclosed multiple pathological lymph nodes above and under the diaphragm with a large intra-abdominal conglomerate residing close to the intestine, with central necrosis. (C, D) CT scan evaluation after two cycles with daratumumab+COEP (cyclophosphamide, vincristine, etoposide and dexamethasone) shows regression of thoracic and abdominal lymph nodes.