THE ONSET OF B-CHRONIC LYMPHOCYTIC LEUKEMIA IN HODGKIN’S DISEASE: A CASE REPORT
Autori:
Nikša Turk, Kajko Kušec, Marija Dominis, Maruska Marusic-Vrsalovic, Branimir Jaksic
Sažetak
Sažetak. Prikazuj emo slučaj bolesnice (79 g.) s patohistološkom dij agnozom Hodgkinova limfoma (l-IL) (stadij IIIB, histološki tip MC) liječene kemoterapijom po protokolu LVPP (6 ciklusa) s dobrim terapij skim odgovorom. Neočekivano, 18 mjeseci nakon postavljanja dijagnoze HL-a javlja se leukocitoza (19,4×109/L) sa 65% limfocita s limfoplazmocitnom diferencij acijom. Imunofenotip ovih stanica tipičan je za B-kroničnu limfocitnu leukemiju (B-KLL) (CD5/CD19+, CD23-, CD38i; uz slabi izražaj monoklonskih lakih lanaca Ä.). Molekulamom analizom preuredbe gena za teški lanac imunoglobulina (IgH) potvrđena je klonalnost limfocita perifeme krvi. Postavljanjem dijagnoze B-KLL nametnulo se pitanje povezanosti dviju neoplazma limfocitnog podrijetla. Molekulamom analizom bioptata limfnog čvora iz vremena postavljanja dijagnoze limfoma nađena je klonska populacija B-limfocita. Time smo nedvojbeno dokazali koegzistenciju dviju bolesti uz klinički jasnu pojavu HL. Tek su naknadne molekularne analize arhivskih materijala, morfološki urednih razmaza perifeme krvi potvrdile postojanje klonskih B-limfocita, bez dijagnostičkih kriterija limfoproliferativne bolesti tipa KLL. Ovakav nalaz etiološki isključuje sekImdamu leukemiju. Kao nepotvrđena hipoteza ostaje mogućnost netipičnog prezentiranja KLL-a u obliku Richterova sindroma s obilježjima HL-a bez limfocitoze od samog začetka. Ovom hipotezom ostavljamo neodgovoreno pitanje radi li se možda u ovom slučaju o različitim kliničkim oblicima iste bolesti.
Summary
Summary. We present a case of a female patient (79 years) with pathohistologic diagnosis of Hodgkin’s lymphoma (HL) (stage IIIB, histologic type MC) for which she was treated with chemotherapy according to LVPP protocol (6 cycles) with good therapeutic response. Unexpectedly, 18 months after HL diagnosis leukocytosis occurred (19.4×109/L) with 65% of lympho- cytes with lymphoplasmocytic differentiation. Immunophenotype of these cells is typical for B-chronic lymphocytic leukemia (B-CLL) (CD5/CD19+, CD23–, CD38±; with weak expression of monoclonal light chains λ). Molecular analysis confirmed clonal immunoglobulin heavy chain gene (IgH) rearrangement of peripheral blood lymphocytes. The diagnosis of B-CLL imposed the question of the connection between two neoplasms of lymphocytic origin. Molecular analysis of lymph node biopsy taken at the time of lymphoma diagnosis revealed clonal population of B lymphocytes. That test undeniably proved coexistence of both diseases from the beginning. The latest PCR analysis of archive peripheral blood smears confirmed B lymphocyte clonality without diagnostic criteria for lymphoproliferative disease of CLL type. This finding etiologically excludes secondary leukemia. The possibility of untypical presentation of CLL in transformation to Richter’s syndrome with morphologic characteristics of HL from the beginning stays unconfirmed. The hypothetical question that remains unanswered is: »Was it one disease in different clinical forms?«
