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.

Follow-up monoclonal gammopathy of undetermined significance in kidney transplant.

OBJECTIVE: The aim of this work was to review the incidence of monoclonal gammopathy of undetermined significance (MGUS) and complications in kidney transplant (KT) patients at the Puerta del Mar Hospital in Cádiz, Spain. This diagnosis was not considered to be a contraindication for transplantation. METHODS: To estimate the incidence of MGUS in KT patients we used the database of our hospital, which included 1,016 patients who received a KT from 1992 to 2012 with a median follow-up of 30 months. The incidence of MGUS in non-transplant patients was estimated from the literature. RESULTS: Out of 1,016 KT patients, 16 developed MGUS; 10 (72.5%) were >50 years old. Two patients developed post-transplantation lymphoproliferative disorders. No cases of progression to multiple myeloma or amyloidosis were seen during immune suppression therapy or after. CONCLUSIONS: MGUS was >100 times more frequent in KT recipients than in the general population (P < .05). But in contrast to MGUS in general population, progression to plasma cell dyscrasia in these patients was absent and its incidence is unknown in KT patients.

Antiphospholipid antibodies are related to portal vein thrombosis in patients with liver cirrhosis.

The pathogenesis of portal vein thrombosis (PVT) in cirrhotic liver patients is not known. PVT has been related to liver dysfunction, neoplasm, hemodynamic factors, and hypercoagulability states. PVT has been reported in patients with antiphospholipid syndrome without liver cirrhosis. Our aim was to find the role of antiphospholipid antibodies (APAs) and coagulation inhibitors in PVT in patients with liver cirrhosis. We present a case-controlled study, matched by age, liver function, and etiology, to discover the role of APAs and anticoagulant protein activity in PVT in cirrhotic patients. We studied 30 cirrhotic patients: 6 of 10 (60%) patients with PVT were APA-positive, whereas only 2 of 20 (10%) in the cirrhotic control group were APA-positive (p < 0.005). Low serum levels of protein C, protein S antithrombin III, and plasminogen were found in cirrhotic patients; and, no differences were found between patients with and without PVT. Significantly lower protein S and antithrombin III levels were found in patients with Child-Pugh class C. Therefore, APAs were related to PVT in cirrhotic patients; but, a lower concentration of coagulation inhibitors was associated with liver dysfunction alone.

[Prevalence and clinical significance of antiphospholipid antibodies in chronic hepatitis C]. / Prevalencia y significado clínico de los anticuerpos antifosfolipídicos en la hepatitis crónica por virus C.

BACKGROUND: To know the prevalence of antiphospholipid antibodies in chronic hepatitis C and their relationship with disease progression. METHODS: One hundred and twenty-eight patients with chronic hepatitis C and 93 healthy controls were enrolled up. We determined platelets, ALT, gamma GT, RNAHCV in serum and liver and non-organ specific antibodies, grade and stage in liver biopsy, risk factors, duration of disease and alcohol intake were also included. Portal hypertension and liver function parameters were studied. Antiphospholipid antibodies (APA): lupus anticoagulant (LA) and anticardiolipin antibodies (ACA) (IgG and IgM) were measured by EIA. Anti-beta 2 glycoprotein I antibodies were also detected by EIA in ACA positive patients. RESULTS: Thirty one out of 128 (25%; 95% CI: 17.8%-33.4%) showed positive antiphospholipid antibodies. Positive ACA-IgG was higher in patients than controls (22% vs 3.2%; p < 0.05), whereas, ACA-IgM was similar (5% vs 3.2%; p = NS), and LA was absent in both groups. ALT levels, viraemia, viral load in liver, platelets, or ANA titre were similar in patients with and without positive ACA-IgG. Risk factors, duration of disease or alcohol intake were not related yet. Patients with staging F1 showed positive ACA-IgG 4 of 44 (9%; 95% CI: 2.5%-21.7%), in staging F2 7 of 39 (18%; 95% CI: 7.5%-33.5%) and in staging F4 17 of 45 (38%; 95% CI: 23.8%-53.5%; p < 0.005). ACA-IgG was significantly related to portal hypertension, Child-Pugh stage and presence of cirrhosis complications. Anti-beta 2 glycoprotein I antibodies were detected in ten (43.5%; CI 95%: 23.2%-65.5%) out of 23 ACA positive patients. CONCLUSIONS: ACA-IgG seems to be associated with chronic hepatitis C, and could play a potential role in fibrosis progression and liver disease in these patients.