Response to azacitidine in patients with chronic myelomonocytic leukemia according to overlap myelodysplastic/myeloproliferative neoplasms criteria.

BACKGROUND: Azacitidine (AZA) is approved for the treatment of high-risk chronic myelomonocytic leukemia (CMML) of myelodysplastic (MD) subtype. Data of response rates using the specific response criteria for this disease are scarce. The aim of this study was to evaluate the response to AZA in patients diagnosed with CMML from the Spanish Registry of Myelodysplastic Syndromes (MDS) applying the overlap myelodysplastic/myeloproliferative neoplasms (MDS/MPN) response criteria. METHODS: We retrospectively studied 91 patients with CMML treated with at least one cycle of AZA from the Spanish Registry of MDS. As it was a real-world study, the response rate was evaluated between cycle 4 and 6, applying the MDS/MPN response criteria FINDINGS: The overall response rate at cycle 4-6 was 58%. Almost half of the patients achieved transfusion independence and one quarter showed clinical benefit, regardless of the CMML French-American-British (FAB) and World Health Organization (WHO) subtypes and CMML Specific Prognosis Scoring (CPSS) risk groups. Toxicity was higher in the MD-CMML subtype. INTERPRETATION: In our series, most CMML patients achieved an overall response rate with AZA according to the overlap-MDS/MPN response criteria regardless of the CMML FAB and WHO subtypes and CPSS risk groups. Thus, AZA may also be a treatment option for patients with the myeloproliferative CMML subtype and those with a lower-risk CPSS, but symptomatic.

The evaluation of left and right ventricular hypertrophy in combined ventricular overload by electrocardiography: relationship with the echocardiographic data.

The electrocardiographic and echocardiographic (M-mode) data were analyzed in 29 patients affected by mitral or combined mitral and aortic valve disease and with hemodynamically documented biventricular overload. No electrocardiographic parameter significantly correlated with the left ventricular internal dimension at end diastole (LVIDd), the left posterior wall (LVPW) thickness and the left ventricular mass (LV mass). A significant correlation was observed between the R/S ratio in V1 and V2, the rV1 and either the end-diastolic right ventricular internal dimension (RVIDs) or the pulmonary artery systolic pressure (PASP). The R/S ratio greater than or equal to 1 in V1 was the most sensitive among the conventional electrocardiographic criteria of right ventricular enlargement. Three groups of patients were selected on the basis of RVIDd and LV mass: Group A included nine patients with right ventricular dilatation and normal LV mass; Group B included ten patients without right ventricular dilatation and with increased LV mass; Group C included ten patients without right ventricular dilatation and with normal LV mass. The R/S ratio in V1 was significantly greater in patients in group A than in those in groups B or C. No electrocardiographic parameter was found to be significantly different between groups B and C. When only LV mass was considered (independently from RVId and PASP), no electrocardiographic parameter differentiated patients with LV mass greater than 203 g from those with LV mass less than 203 g. We conclude that in patients with biventricular overload secondary to acquired valvular disease: 1) the electrocardiographic diagnosis of left ventricular enlargement is unreliable; and 2) the R/S ratio in V1 is the most sensitive parameter to predict right ventricular enlargement or severe pulmonary hypertension.

[Contribution of bidimensional echocardiography to the diagnosis of left atrial enlargement]. / Contributo della ecocardiografia bidimensionale nella diagnosi di ingrandimento atriale sinistro.

In 64 consecutive subjects (age range 17-69 years) we compared the antero-posterior (A-P) size of the left atrium estimated by M-mode and wide-angle two-dimensional echocardiography (short and long axis parasternal views). M-mode A-P diameter correlated both with short (r = 0.82) and with long (r = 0.76) axis A-P diameter. Subsequently we quantitatively analysed by two-dimensional echocardiography A-P, cranio-caudal (C-C) and latero-lateral (L-L) left atrial dimensions in 20 patients with mitral valve disease (age range 24-66 years) and in 24 normal subjects of comparable age range. Subjects with mitral valve disease had significantly higher A-P (P less than 0.001), C-C (P less than 0.02) and L-L (P less than 0.001) left atrial dimensions than normal controls. Both in normals and in mitral valve disease patients C-C was the view that showed the largest atrial diameter. Left atrial shape was however more spherical in mitral valve patients. Of these 7/20 (35%) had abnormal A-P diameter compared to the normal subjects. Patients with abnormal L-L and C-C left atrial dimensions were 9/20 (45%) and 7/20 (35%). Five of the patients with increased L-L size had a normal A-P diameter. Our results demonstrate the usefulness of wide-angle two-dimensional echocardiography in the assessment of the left atrial enlargement, especially in patients with negative or borderline M-mode echocardiographic data.