Acute myeloid leukemia with cup-like blasts and FLT3-ITD and NPM1 mutations mimics features of acute promyelocytic leukemia: a case of durable remission after sorafenib and low-dose cytarabine.

Some previous researches raised the possibility of a novel acute myeloid leukemia (AML) entity presenting cup-like cytomorphology with mutations of both FLT3 and NPM1 or one of them. However, the clinical implications of this subtype remain unknown. We describe a 63-year-old patient belonging to this distinct AML subtype, who presented similar features of acute promyelocytic leukemia (APL) including nuclear morphology, negative for CD34 and HLA-DR, and abnormal coagulation. He had no response to both arsenic trioxide and CAG regimen (cytarabine, aclarubicin, and G-CSF). Given that the patient carried the FLT3-ITD mutation, we switched to a pilot treatment of FLT3 inhibitor sorafenib combined with low-dose cytarabine (LDAC). To date, the patient achieved durable complete remission over 58 months. These findings suggest that AML with cup-like blasts and FLT3-ITD and NPM1 mutations mimic APL, and the prognosis of this subtype may be improved by sorafenib combined with LDAC.

[The cytogenetic characteristics of 178 acute myeloid leukemia patients].

OBJECTIVE: To explore the cytogenetic characteristics of acute myeloid leukemia (AML) patients. METHODS: The karyotype analysis was performed in 178 AML using the short-term culture of bone marrow cell and G-banding technique. RESULTS: Among the 178 patients, 171 had enough metaphases for analysis and 128 (74.9%) had clonal karyotypic abnormalities. Twenty-seven patients were secondary to myelodysplastic syndrome (MDS-AML), with 25 (92.6%) patients carrying clonal karyotypic abnormalities. Among the remaining 144 patients of de novo AML, 103 (71.5%) had clonal karyotypic abnormalities. The rate of abnormal clonal karyotype was higher in MDS-AML than that of de novo AML (P = 0.021). Among the 171 patients, 41 (24.0%) were in favorable risk group, 80(46.8%) in intermediate risk group and 50 (29.2%) in adverse risk group. t(15;17) was the most common chromosomal aberration. The majority intermediate risk chromosomal aberration was normal karyotype. The most common cytogenetic abnormality among adverse group was a complex karyotype. Adverse cytogenetic aberrations, such as -5/5q-, -7/7q-, frequently occurred in conjunction with one another as part of a complex karyotype. Totally 75 patients were 60 years or older, among them, 16.0% were in favorable risk group, 48.0% in intermediate risk group and 36.0% in adverse risk group. Among 96 younger patients, 30.2% were in favorable risk group, 45.8% in intermediate risk group and 24.0% in adverse risk group. The rate of favorable risk chromosomal aberration was lower in elder patients than in younger (P = 0.031). The rate of adverse risk chromosomal aberration and the rate of monosomal karyotype were higher in MDS-AML than in de novo AML patients (P < 0.001). CONCLUSIONS: The most common favorable, intermediate and adverse chromosomal aberrations were t(15;17), normal karyotype and complex karyotype respectively. The karyotype was poor in MDS-AML and elder AML patients.

[A clinical study of 103 idiopathic thrombocytopenic purpura].

OBJECTIVE: To investigate the prevalence by age, response to different therapies and outcome in newly diagnosed idiopathic thrombocytopenic purpura (ITP). METHODS: ITP patients who were hospitalized from July 1992 to December 2006 and followed up with telephone were retrospectively analyzed. RESULTS: 103 patients with ITP were investigated. The time of follow-up was between 2 months to 15years. Male:female = 35:68. The rate of patients over 60 years old was 34.0% (35/103). 59 patients were sensitive to adrenocorticosteroid and 4 patients under going splenectomy achieved a normal platelet count. In those immunosuppressive agents: including vincristine, cyclophosphamide, azathioprine and cyclosporin A(CsA) used in the present series, CsA was shown to be more effective. It could increase the platelet count when given together with prednisolone, the effective rate was 81.3% (26/32). Severe side effects like kiney function failure were not found in CsA treated patients so the use of geug in ITP would be recommended. There were 2, 1 and 1 ITP patients progressing respectively to Sjogren' s syndrome, systemic lupus erythematosus and lymphoma. 7 patients died, 1 patient died of cerebral bleeding, 2 brain infarction, 3 malignant neoplasm and 1 nephrosis The refractory rate of ITP is 17.2% (10/58). CONCLUSIONS: The morbidity in older people is high. The mortal bleeding in ITP is low. Treatment should be tailored to the individual patient.

[Effects of ferrous sulfate supplementation on bone marrow hemopoiesis in rats].

OBJECTIVE: To explore the influences of different dosage ferrous sulfate supplements on bone marrow hemopoiesis in rats. METHODS: Female weaning Wistar rats were fed with an iron deficient diet (< 10 mg/kg diet) until the level of hemoglobin of rats was lower than 100 g/L. Rats (n = 50) were randomly divided into five groups according to the levels of hemoglobin and body weight, iron deficiency control (ID), daily low iron diet supplement (LDs), daily high iron diet supplement (HDs), weekly low iron supplement (LWs), and weekly high iron supplement (HWs). RESULTS: After 12 weeks, bone marrow stainable iron was seldom in ID group, and ample in supplement groups. The proportions of iron staining of bone marrow smear in supplement groups were more than 30%. Bone marrow cells in all rats were hyperplastic or active hyperplastic. CONCLUSIONS: Daily high iron supplement or once weekly high iron supplement were safe to bone marrow hemopoiesis in rats.

[Monosomal karyotype among adult acute myeloid leukemia: clinical characteristic and prognostic analysis].

OBJECTIVE: To explore the clinical characteristics and prognostic value of monosomal karyotype (MK) patients in adult acute myeloid leukemia (AML). METHODS: We retrospectively studied 45 patients of MK⁺ in newly-diagnosed adult AML in our center from Oct 2000 to Dec 2012. Clinical characteristics, cytogenetic data and prognostic features were analyzed in the cohort of MK⁺ patients. RESULTS: MK was found in 45 patients (19.0%) of 237 newly-diagnosed adult AML with cytogenetic data available at diagnoses. Among these 45 cases, there were 28 male (62.2%) and 17 female (37.8%). Median age of MK⁺ patients at diagnose was 58(18-91) years old. The presence of -5(31.1%) and -7(17.8%) were the most common chromatid among MK⁺ AML patients. MK was much more prevalent among elderly patients. Among AML patients, the proportions of MK⁺ patients younger than 30, 30 to 59 and older than 60 years old groups were 11.5%, 17.7% and 22.4%, respectively. There was no difference between MK⁺ and MK⁻ patients in gender distribution (P=0.545). There was also no difference between MK⁺ and MK⁻ patients in the distribution of FAB castigation (P=0.239). Median survival of MK⁺ AML patients was 6.5 months. Cumulative 5-year overall survival (OS) of was 5.2%. Forty-three MK⁺ patients (43/45, 95.6%) also had a complex karyotype (CK). Two cases that did not meet the CK had not achieved complete remission (CR), and died within 6 months. There were 12 patients who were CK⁺ in 192 MK⁻ patients. The differences of OS and CR rates between MK⁺CK⁺ patients and MK⁻CK⁺ were statistically significant (P<0.05). CONCLUSION: The increased detection rate of MK with age was associated with lower CR and OS in AML patients.