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Key Clinical Message Nontuberculous mycobacteria infrequently cause disseminated infections in immunocompetent hosts. However, they are increasingly being recognized in immunocompromised patients. We present the case of a 40-year-old HIV-positive male presenting with lymphadenopathies and pancytopenia in whom disseminated infection, with bone marrow involvement by Mycobacterium genavense ( M. genavense) was diagnosed. [ABSTRACT FROM AUTHOR]

: BackgroundDifferent flow cytometric methods have been developed to derive absolute CD34+ cells in predicting transplant outcome. Two techniques for preparing cells for quantification of CD34+ cells were compared.: MethodsEnumeration of CD34+ cells in 16 samples of bone marrow (BM) and in 29 samples of mobilized peripheral blood stem cells (PBSC) obtained by leukapheresis was assessed simultaneously by single-platform (ProCOUNT kit) and dual-platform (Milan protocol) approaches within the first 3 h of collection.: ResultsAbsolute number of CD34+ cells obtained in PBSC and BM using single- and dual-platform methods showed high determination coefficients as follows: for PBSC, slope = 1.0515 ± 0.048, y-intercept = 88.638 ± 52.45, and r2 = 0.941, and for BM, slope = 1.0203 ± 0.093, y-intercept = 122.25 ± 20.65, and r2 = 0.878. There were no statistically significant differences in absolute number of CD34+ cells from PBSC between single-platform (mean 575/μL, range 70–3,683/μL) and dual-platform (786/μL, range 51–3,804/μL) assays. In contrast, absolute number of CD34+ cells from BM was significantly lower (p = 0.0002) when enumerated by ProCount kit (135/μL, 14–758/μL) than with dual-platform method (260/μL, 74–889/μL).: ConclusionsBoth approaches can be used indistinctly to estimate absolute number of CD34+ cells in PBSC but not in BM. [Copyright &y& Elsevier]

Background: In Mexico, the frequency of thromboembolic events associated to paroxysmal nocturnal hemoglobinuria is 3%; a clone size > 50% in granulocytes has been associated with a higher risk of thromboembolic events., Methods: Between 2001 and 2012, 40 patients with paroxysmal nocturnal hemoglobinuria were studied. In 12 cases anticoagulant, procoagulant, and fibrinolytic pathways were analyzed., Results: Only two of 40 patients (5%) developed a thromboembolic event over a 25.5-year follow-up period. From 12 patients, 91.7% had a paroxysmal nocturnal hemoglobinuria clone > 50% in granulocytes and 83.3% a clone > 50 % in monocytes. Five of 12 cases had elevated FV levels and four showed increased FVIII, von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and FX. Protein S and protein C were decreased in nine and three patients, respectively. Only antithrombin correlated positively with paroxysmal nocturnal hemoglobinuria clone size in monocytes (p = 0.0442), whereas von Willebrand factor ristocetin cofactor correlated negatively with lactic dehydrogenase levels (p = 0.0186). No statistically significant associations were recorded with all other factors., Conclusion: The low frequency of thromboembolic events in Mexican patients could partly be explained by the associations between anticoagulant system (antithrombin) with paroxysmal nocturnal hemoglobinuria monocyte clone size, and procoagulant system (von Willebrand factor ristocetin cofactor) with lactic dehydrogenase levels.

Background: Myelodysplastic syndromes (MDS) are clonal diseases of hematopoietic cells. The International Prognostic Scoring System (IPSS) is the risk scale most employed in MDS. Cyclosporin A (CsA) has been used in the treatment of cytopenias in MDS., Objective: To evaluate hematologic response and identify response predictive factors in adults with MDS treated with CsA., Material and Methods: Patients with MDS diagnosed according World Health Organization (WHO) classification were recruited from January 1997 to June 2012. All patients were classified with IPSS, IPSS revised (IPSS-R),WHO Prognostic Scoring System (WPSS), and WPSS revised (WPSS-R) risk scales. Cyclosporin A was administered orally at a dose of 5 mg/kg/day. Hematologic response was evaluated following the International Working Group for MDS (2006 version) criteria., Results: Inclusion criteria were met by 32 patients. Median age was 56.5 years, with a median follow-up of 3.1 years. Hematologic response was 56.2% and erythrocyte independence transfusion was found in 42.9% of patients. Age,hemoglobin level, and WPSS at diagnosis were independent predictive factors for CsA response. Survival was longer in responder than in nonresponder CsA patients (p=0.06)., Conclusions: Cyclosporin A induced hematologic response in >50% of patients with MDS aged <57 years, with Hb<8 g/dl and low WPSS at diagnosis.

Background: in Mexico published casuistry concerning hairy cell leukemia (HCL) is limited., Objective: to describe the therapeutic response and survival of patients with HCL attended in a third level public institution., Methods: patient's data with HCL diagnosis registered between January 1989 - December 2009 were analyzed., Results: twenty three patients fulfilled HCL diagnosis criteria. Median age was 44 years (range 23-75 years) and median follow-up of the cohort was 1,877 days (range 1-8,462 days). First line treatment varied along time finding complete response (CR) and partial response (PR) rates of 77.3 and 18.2%, respectively. Of all therapeutic modalities employed cladribine induced the highest response rate. Survival at 1,877 days was 82.6%. At last follow-up 65.2% of patients remain alive, 13 in CR and 2 in PR; 4 died (CR = 2, PR = 1, active disease = 1) and 4 were lost during follow-up., Conclusion: this study which included more patients than previous single-institution Mexican series confirm the chronic clinical behavior of HCL and that purine analogs are corner stone in the treatment of patients suffering HCL.

Introduction: Acute myeloid leukemia (AML) comprises a group of diseases with different biologic characteristics; despite knowledge improvements, these are not reflected in long term survival., Objective: To describe characteristics of adults with AML in a hospital of Mexico City, their treatment response, complications and to evaluate survival related factors., Material and Methods: Cohort study. Between January 2003 and July 2008, patients with AML diagnosis were included (except promyelocitic). Treatment protocols used: 3 + 7, high doses of cytarabine and autologous bone marrow transplant as consolidation therapy., Results: 53 patients were included. Median age: 44 years (15-79). At diagnosis: tumor lysis syndrome in 4/ 53 (7.5%), 3/51 (5.9%) with altered liver function test and hyperleukocytosis in 8/53 (15.1%). 46 patients had available cytogenetic and this was successful in 28/46 (60.8%), 12/28 (42.8%) had adverse cytogenetic; 16/28 (57.1%) intermediate risk and none was favorable. There were 2 losses during follow up, 7 patients did not receive chemotherapy with curative intent and 1 died at diagnosis. 43 patients received 3 + 7, 13.9% died during aplasia, complete remission was achieved in 27/43 (62.7%) and 10/43 (23.2%) were refractory to treatment. A second induction attempt was required in 39.5% (17/43). Median disease free survival (DFS) was 491 days (366-615), with a median follow up of 993 days (105-1744). The median overall survival (OS) was 531 days (312-749). Aplasia related mortality decreased (p = 0.09) between the actual cohort (13.9%) and the historical cohort (37%)., Conclusions: Long term survival in AML patients remains poor despite improvements in diagnosis, classification, and treatment. In our institution, it is required to improve induction protocols and cytogenetic analysis in order to adequately choose the group of patients that could be benefit from stem cell transplant.

Introduction: Despite therapeutic advances, acute lymphoblastic leukemia (ALL) in adults remains a disease with poor long term outcome and survival rates. Developing countries lack of information about this disease. On the other hand, infections are frequent complications related to mortality and some research studies do not show accurate rates of septic shock or other related factors., Objective: To describe characteristics of adults with acute lymphoblastic leukemia, response to treatment, complications and to evaluate further survival related factors and to compare our experience with other reports of literature., Material and Methods: Between September 2003 to November 2007, the entire cohort of patients with diagnosis of ALL was included. The treatment regimens used were MDACC HyperCVAD (HCVAD) and 0195 (institutional regimen)., Results: Of 40 patients included with the diagnosis of ALL, 92% was B phenotype and 8%, T phenotype, with a median age of 27 years. The median follow up was 28.5 months. Initially, 14% showed central nervous system infiltration; of 51% with available cytogenetics, 16.7% was Philadelphia chromosome positive. There were 36 patients who received treatment: 13 received HCVAD and 23 the 0195 protocol; 78% achieved global complete remission, 85% for the patients with HCVAD and 74% with 0195. The induction death rate was 2.8%. The median disease-free survival was 11.6 months (IC 95%, 2.5-20.8 months) and overall survival was 15 months (IC 95%, 10.6-19.4 months). In 95% of patients, no prophylactic antibiotic therapy was used and treatment related death was 8.4% (2.8% during induction and 5.6% during the rest of treatment). Factors associated with worse survival rate were hyperleukocytosis, T phenotype and lack of early complete remission. During induction, grade 3 to 4 non hematopoietic toxicity was 17%. Incidence of neutropenic febrile episodes was 61% and septic shock was 11%., Conclusions: With HCVAD, we observed worse complete remission, disease-free survival and overall survival rates compared with the original MDACC reports. Chemotherapy related death rates are similar to other early reports, despite prophylactic antibiotic was not used during myelosuppression.

Thalidomide, an immunomodulatory and antiangiogenic agent, is useful in the treatment of some hematologic and oncologic diseases. Up to 6.8% of thalidomide-treated patients present bradycardia. Herein the incidence of thalidomide-associated bradycardia in patients with hematologic diseases treated in a single institution is reported. In a 34-month period, 33 patients with different hematologic diseases (multiple myeloma [MM], 20; myelodysplastic syndrome, eight; Waldenström macroglobulinemia, two; non-Hodgkin's lymphoma, two; malignant histiocytosis, one) were treated with thalidomide. Of them, five (15.1%) had bradycardia, all with MM. Bradycardia was detected with a daily thalidomide dose ranging from 100 to 300 mg and the time patients received thalidomide before cardiac event went from one to 18 months. In all affected cases the electrocardiogram showed sinus bradycardia with cardiac frequency between 32 to 48 beats per minute. Time to normal cardiac beat recovery ranged from 12 to 21 days after thalidomide discontinuation. There were no fatalities due to thalidomide-associated bradycardia. It is concluded that: a) thalidomide-associated bradycardia was detected only in patients with MM, b) herein the incidence of bradycardia was higher as compared with other series, and c) in patients with MM thalidomide therapy must be prescribed with caution particularly in those with cardiovascular diseases of any etiology.

Objective: To describe the frequency and compare the clinical characteristics, treatment response, survival and hematologic, immunophenotypic, cytogenetic, and histologic findings in adult patients with acute megakaryoblastic leukemia (AMegL) and megakaryocytic blast crisis of chronic myeloid leukemia (MegBC-CML)., Material and Methods: The records of patients with AMegL and MegBC-CML attended in our institution between July 1993 and December 2000 were revised. Megakaryocytic lineage was established by the presence of one or more megakaryocyte/platelet associated antigens (CD41, CD42b, and CD61) in > 20% blast cells., Results: In 90 months, 277 patients with acute leukemia were admitted and 25 with chronic myeloid leukemia (CML) in blast crisis (BC) were identified. Twelve of 125 patients (9.6%) with acute myeloid leukemia were AMegL and 32% of cases with CML-BC were MegBC-CML. Leukemic cells of patients with AMegL expressed more frequently CD15 antigen than blast cells of those with MegBC-CML (83% and 37.5%; p < 0.05). In contrast, blast cells expressing myeloperoxidase were present in 50% and 10% of cases with MegBC-CML and AMegL, respectively (p < 0.05). Only one patient in each group obtained remission. Although median survival in patients with AMegL was lower (70 days) than in those with MegBC-CML (175 days) the difference did not reach statistical significance., Conclusion: AMegL and MegBC-CML differ in some clinical and laboratory characteristics and are diseases with poor treatment response and short survival.

Objective: P-glycoprotein (P-gp) expression has been widely observed in normal and neoplastic cells. The physiologic role of P-gp involves hormone and metabolite secretion, bacterial product detoxification, and transport of several drugs to the extracellular space. Multidrug resistance-1 is characterized by drug extrusion through P-gp, reducing the intracellular levels of drugs and diminishing their pharmacological effects. Treatment of immune thrombocytopenic purpura (ITP) includes agents that are substrates of P-gp; hence, the objective of this study was to analyze the functional activity of P-gp in lymphocytes from patients with ITP., Patients and Methods: 30 ITP patients (9 refractory, 5 dependent, 14 responders to treatment, and 2 with stable disease) and 25 healthy controls were studied. Peripheral blood mononuclear cells were isolated by gradient centrifugation and incubated with daunorubicin (a fluorescent drug extruded by P-gp). Functional activity of P-gp was analyzed by flow cytometry. Results were expressed as the percentage of lymphocytes able to extrude daunorubicin., Results: ITP patients showed an increased number of lymphocytes with P-gp activity (mean=12.3%+/-16%) when compared to controls (mean=0.87%+/-0.72%) (p<0.05). P-gp function was higher in the refractory group (median=9.4%) than in the treatment-dependent (median=5.4%), responder (median=6.4%), and stable disease (median=5.2%) groups, although no statistical differences were found among them., Conclusion: Enhanced P-gp activity in ITP may be related to an unfavorable clinical outcome and poor response to treatment. Furthermore, P-gp function might affect therapeutic requirements for disease control.