‘Lymphoid’ blast crisis of chronic myeloid leukaemia is associated with distinct clinicohaematological features

Abstract

It has been suggested that in blast crisis (BC) of chronic myeloid leukaemia (CML) the clinical and laboratory features of patients with 'lymphoid' phenotype differ from those of patients with non-lymphoid BC. In order to assess any differences, 97 patients consecutively diagnosed with BC that followed a known chronic phase of CML were analysed. 19 patients had 'lymphoid' BC: in 17 the blasts expressed a B-lineage phenotype: in the remaining two they corresponded to T lymphoblasts. Four cases of B-lineage phenotype BC were considered as biphenotypic, due to the co-expression of myeloperoxidase and one or two other myeloid markers (CD33, CD13 and CD68) on the blast cells; in the other six cases of B-lineage BC the blasts expressed one or both of the myeloid markers CD33 (n = 4) and CD13 (n = 3). Patients with 'lymphoid' BC seldom had an accelerated phase prior to BC (1/19 v 36/78 with non-lymphoid BC, P = 0.002), had less frequent splenomegaly (9/19 v 59/78, P = 0.03) and hepatomegaly (5/19 v 45/78, P = 0.02) and showed a higher degree of marrow blast infiltration (mean value 74 +/- 24% v 38 +/- 23%, P < 0.0001), lesser blood basophilia (2.2 +/- 2.5% v 8.2 +/- 7.8%, P < 0.0001), and higher serum albumin levels (P = 0.001) than those with non-lymphoid BC. 13 patients with 'lymphoid' BC (68.4%) showed a favourable response to chemotherapy regimens including vincristine and prednisone and, overall, 'lymphoid' BC patients survived significantly longer than the remainder (median survival 12 months v 4.7 months, P = 0.006). These results indicate that 'lymphoid' BC of CML has a distinct clinicohaematological profile and confirm the better prognosis of such patients.

The spectrum of adult B‐lymphoid leukemias with BCR‐ABL: Molecular diagnostic, cytogenetic, and clinical laboratory perspectives

“…Furthermore, peripheral eosinophilia and basophilia were also uncommon in the lymphoblastic CML cases. Other studies have shown that lymphoid blast crisis, compared with myeloid, is associated with less frequent splenomegaly, less peripheral basophilia, and a lower frequency of additional clonal abnormalities, and thus are consistent with our findings presented here [14,15]. Furthermore, lymphoid blast crisis is usually associated with a better survival and response to therapy [16].…”

Extramedullary T cell lymphoblastic transformation of chronic myeloid leukaemia successfully treated with matched unrelated donor bone marrow transplantation

“…6,7 Achieving a long-lasting remission, however, seems unlikely with this approach. As the favourable treatment-response associated with lymphoid BC is brief with a median survival of 9 months after diagnosis, plans for definitive strategies such as allogeneic BMT should be implemented rapidly.…”

Reporte del Primer Consenso Colombiano de Citometría de Flujo para el estudio de trastornos hematológicos

“…Por el contrario, la citometría de flujo es útil para realizar el recuento de blastos y diferenciar entre las fases crónica, acelerada y de crisis blástica, y para determinar el inmunofenotipo de los blastos en la crisis blástica (mieloide en 60% a 70% de los casos versus linfoide en 20% a 30%) (35). No obstante, la reciente introducción de nuevas herramientas técnicas basadas en la utilización de perlas inmunomarcadas para detectar la proteína de fusión del BCR-ABL, abre las puertas, como nueva prueba muy sensible y específica, para confirmar o descartar con gran rapidez la presencia de la t(9;22) en casos en los que existe una sospecha diagnóstica razonada de leucemia mieloide crónica (39) La citometría de flujo también es útil en la detección de mastocitosis sistémica, relacionada con síndromes mieloproliferativos o con neoplasia mieloide de otro tipo, como leucemia mieloide aguda o síndromes mielodisplásicos, al evaluar la expresión del marcador CD117 para la identificación de los mastocitos en su conjunto, que en las mastocitosis presentan de forma aberrante expresión de CD25 y CD2 (36,37).…”