Myeloablative Dose of Busulfan and Fludarabine Combined with In Vivo T Cell Depletion Is Safe and Effective Conditioning for Acute Myeloid Leukemia and Myelodysplastic Syndrome Patients.

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative strategy for acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS). The prediction of transplantation-related mortality (TRM) using the Hematopoietic Cell Transplantation Comorbidity Index (HCT-CI) score and an arbitrary upper age limit of 55 years for administering myeloablative conditioning (MAC) are common strategies to ensure a safe procedure. The use of reduced-toxicity conditioning regimens is an additional approach to providing safe and effective myeloablation. Herein we report the outcome of AML and MDS patients conditioned with fludarabine and a myeloablative dose of busulfan (FB4) stratified by age and HCT-CI score. The primary objective was overall survival (OS) for patients age ≥55 years. Secondary objectives were total OS, TRM, graft-versus-host disease (GVHD), and GVHD, relapse-free survival (GRFS). The 2 year OS was 72% in patients age <55 and 51% in patients age ≥55. In patients age ≥55 with an HCT-CI <2, the estimated 2 year OS was 64%, with median OS not reached. In those with HCT-CI ≥2, the 2-year OS was 43%, with a median OS of 14 months. The total cumulative incidence of relapse was 30% regardless of age or HCT-CI score. FB4 conditioning regimen offers a high rate of prolonged remission with a relapse rate similar to that reported in previous studies. These positive outcomes suggest that this conditioning platform can be offered to patients age ≥55 years in the absence of comorbidities, and that age should not be the sole determinant of conditioning intensity.

De novo lymphoid blastic phase chronic myeloid leukemia: report and contemporary discussion.

OBJECTIVE: We herein describe two cases of de novo lymphoid blastic transformation in patients with no history of chronic-phase chronic myeloid leukemia (CP-CML), both of whom were labeled initially as Philadelphia positive B-Acute Lymphoblastic Leukemia (B-ALL). METHODS: The first patient was an 18-year-old male who presented with subjective fever, intentional weight loss, generalized fatigue, and headache. Investigations showed leukocytosis (312 × 10^3/ul), thrombocytopenia and anemia. Flowcytometry was consistent with B-ALL, with aberrant expression of CD13 and CD33. He was found to be positive for BCR::ABL by FISH, and karyotype confirmed the presence of the Philadelphia chromosome. He received a pediatric-inspired regimen and achieved remission with negative measurable residual disease (MRD) by flowcytometry, however with persistent cytogenetic abnormality using FISH for BCR::ABL. FISH abnormality was confirmed to be in the myeloid compartment using myeloid segregated FISH, reclassifying the disease to de novo lymphoid blastic phase CML. The second patient was a 52-year-old male who presented with fever and shortness of breath. Bilateral cervical lymphadenopathy and hepatosplenomegaly were identified on examination, and investigations showed leukocytosis (371 × 10^3/ul), anemia, and thrombocytopenia. BCR::ABL rearrangement was identified by FISH, molecular testing, and confirmed with karyotype. He was treated with Mini-CVD and Ponatinib, achieved complete remission with negative MRD by flow cytometry, however molecular studies showed BCR-ABL1 level at 58% IS indicating a persistent cytogenetic abnormality. RESULTS: De novo lymphoid blastic-phase CML can therefore be difficult to differentiate from Philadelphia positive B-ALL due to their overlapping clinical and laboratory picture, implying the need to do myeloid compartment evaluation at the time of diagnosis. CONCLUSION: With recent progress in the treatment of Philadelphia positive B-ALL, including the role of transplant with the use of novel agents, a better characterization of this disease entity in retrospective and prospective trials is warranted.

Role of 18F-Fluorodeoxyglucose-Positron Emission Tomography/Computed Tomography Imaging in the Prediction of Prognosis in Patients With Indolent Lymphoma: Prospective Study.

BACKGROUND: The role of fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) in indolent lymphoma has been minimally studied. OBJECTIVE: This study aims to assess the value of FDG-PET/CT in predicting the prognosis of indolent lymphoma. METHODS: We prospectively recruited 42 patients with indolent lymphoma. A total of 2 patients were excluded, and 40 underwent baseline PET/CT and follow-up at various time points. A total of 9 patients were observed only, 7 received 4 doses of rituximab alone, and 24 received chemoimmunotherapy. Metabolic response on follow-up PET/CT was assessed using the maximum standardized uptake value (SUVmax) and Deauville criteria (DC). We aimed to obtain the best SUVmax and DC to predict optimal survival rates, risk stratification, and optimize therapeutic strategies. The mean follow-up from the initial diagnosis was 33.83 months. RESULTS: SUVmax <4.35 at interim PET/CT provided the best discrimination, with a progression-free survival (PFS) of 100% and a median survival time of 106.67 months compared with SUVmax ≥4.35 (P=.04), which had a PFS of 43.8% and a median survival time of 50.17 months. This cutoff was also valuable in predicting overall survival at baseline, that is, 100% overall survival with baseline SUVmax <4.35, versus 58.4% for SUVmax ≥4.35 (P=.13). The overall survival of patients with a baseline DC score <3.0 was 100%, with a median overall survival of 106.67 months. CONCLUSIONS: We demonstrated the utility of PET/CT in indolent lymphomas. SUVmax (<4.35 vs ≥4.35) on interim PET/CT performed best in predicting PFS.

An Intriguing Case of Eosinophilia with FIP1L1/PDGFRA Rearrangement Who Presented as Thrombotic Thrombocytopenic Purpura.

Myeloid neoplasm with eosinophilia and FIP1-like-1-platelet-derived growth factor receptor-alpha (FIP1L1-PDGFRA) rearrangement is a multi-organ disease with diverse clinical presentation. Thrombotic thrombocytopenic purpura (TTP) is characterized by the concomitant occurrence of often severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic organ damage. To our knowledge, only one case of eosinophilia with FIP1L1-PDGFRA rearrangement presented as a case of thrombotic thrombocytopenic purpura reported in the literature. We herein report a case of a young male patient with hypereosinophilic syndrome and FIP1L1-PDGFRA rearrangement who presented with asthma, transient ischemic attacks (TIA), and confusion. He had an acquired TTP that was successfully treated with plasma exchanges (PLEX), corticosteroids, rituximab, and later with the addition of imatinib mesylate (Gleevec, Novartis). He remains in complete remission on imatinib 100 mg daily for more than 28 months of follow-up.

Bilateral peritonsillar abscess: A case report and pertinent literature review.

INTRODUCTION: The peritonsillar space is defined as the area between the palatine tonsillar capsule medially and the superior pharyngeal constrictor muscle laterally. Unilateral peritonsillar abscess (PTA) is more common than the bilateral condition. To the best of our knowledge, only 13 cases of bilateral PTA have been reported worldwide; this is the first case reported from Saudi Arabia. PRESENTATION OF CASE: We present a case involving a 16-year-old boy who presented to the emergency department with a5-day history of sore throat, dysphagia, trismus, drooling, and a muffled "hot-potato" voice, but with no symptoms of airway compromise. After several examinations, we diagnosed bilateral PTA. We performed an incision and drained the pus from both sides, which was positive for Streptococcus pyogenes. Postoperatively, the patient improved dramatically, and tonsillectomy was scheduled for 6 weeks later. DISCUSSION: The difficulty in diagnosing bilateral PTA stems from the fact that it does not present with the usual clinical characteristics as unilateral PTA. These symptoms include asymmetry of the tonsils and palate, deviation of the uvula, and unilateral otalgia. As these cases carry the risk of impending airway compromise, we recommend airway management as the first step in treating such cases. CONCLUSION: Based on our case and literature review, we recommend airway management as the first step, followed by further examinations, especially contrast-enhanced computed tomography, in cases of bilateral PTA. This condition lacks the hallmark of unilateral disease and may mimic many conditions, which will have different management approaches.

Effectiveness of stapedotomy in improving hearing sensitivity for 53 otosclerotic patients: retrospective review.

BACKGROUND: Otosclerosis is a common cause of progressive hearing impairment that causes fixation of the stapes. Surgical intervention is the preferred treatment approach to ameliorate the conductive hearing loss associated with stapedial otosclerosis. However, given that it is a difficult and delicate procedure, the surgery may fail for a number of reasons. Therefore, it is very important to evaluate the success rate of the surgical approach used in each regional center. OBJECTIVE: To examine the effectiveness of stapedotomy in improving hearing sensitivity for otosclerotic patients at King Abdul Aziz University Hospital in Riyadh. DESIGN: Retrospective chart review with an analysis of pre- and postoperative surgical treatment. SETTING: Tertiary referral otolaryngology clinic. PATIENTS AND METHODS: All cases who underwent stapedotomy between 1997 and 2009 were retrospectively reviewed. Preoperative and postoperative audiometric assessments were conducted using conventional pure tone audiometry. Differences were analyzed by two-way repeated measures ANOVA. MAIN OUTCOME MEASURE(S): Pre- and postoperative pure tone thresholds for air and bone conduction. RESULT: Fifty-three patients underwent stapedotomy. Stapedotomy yielded significant improvements in mean (SD) postoperative air-conduction thresholds of about 18.7 (11.7) dB (P < .0001) and mean (SD) post.operative bone-conduction thresholds of about 2 (7.2) dB (P < .05). Additionally, a significant correlation was found between improvement in air-conduction thresholds and the size of preoperatve air-bone gap (P < .01) About 70%of patients achieved an air-bone gap of 20 dB or better. None of the cases examined in this study exhibited sensorineural hearing loss or adverse complications following stapedotomy. CONCLUSIONS: Stapedotomy is a safe and effective treatment option for patients with otosclerosis. Given that the majority of participants in this study exhibited mixed hearing loss preoperatively, the results further suggest that stapedotomy can also be effective in improving thresholds for these patients. LIMITATIONS: The sample size was relatively small.

Modified Baha Punch Technique: Least Invasive, Shortest Time and No Suturing.

To describe a new Baha surgical procedure (Modified Punch Technique) with a smaller punch hole, a minimal incision and the use of the hydroxyapatite-coated abutment (BIA400). Retrospective chart review. Patients were implanted between 2012 and 2013 at King Abdulaziz University Hospital (Kingdom of Saudi Arabia). Eligible for initial or replacement bone-anchored hearing system; includes six patients (four adults, two children). Baha surgery using the Punch Method is described. The mean surgical time was 12.33 min (range 6-30), without the single outlier, it was 8.8 min. Bleeding was significant but easily controlled in two patients only. Depending on the postauricular subcutaneous thickness, the sizes of abutment used were 6, 8, 10 or 12 mm. None of the patients experienced severe pain. The postoperative follow-up period was 7 months for one patient, 5 months for three patients, and 3 months for two patients. First report of combined Modified Punch Technique with the BIA400 abutment is minimally invasive, reduces surgical time and results in faster healing and reduced likelihood of postoperative numbness.

Impedance cardiography: noninvasive assessment of hemodynamics and thoracic fluid content during bariatric surgery.

BACKGROUND: The effects of pneumoperitoneum (ppm) on hemodynamic parameters during bariatric surgery were investigated using the impedance cardiography monitor. METHODS: 11 patients with BMI 46.5+/-10 kg/m2 (range 38.9-60.8 kg/m2) underwent laparoscopic adjustable gastric banding under general anesthesia. Besides routine monitoring, the impedance cardiography (ICG) monitor was used to monitor cardiac output (CO), cardiac index (CI), systemic vascular resistance (SVR), and thoracic fluid content (TFC). Data were recorded at three stages: A) before ppm, B) during ppm, and C) after gas deflation. One-way analysis of variance (ANOVA) was used to analyze differences of the data before, during and after ppm, and post-hoc (Bonferoni test) for multiple comparisons of the data obtained. For all comparisons, P<0.05 was considered significant. RESULTS: There were significant low mean values of heart rate (HR), CO and CI at stage B compared to stage A (P<0.05). The mean values of TFC at stages A, B, and C were 30.48 +/- 4.69, 29.74 +/- 2.86 and 31.72 +/- 4.93 k/Ohm respectively, with a non-significant relationship (P>0.05). The mean values of SVR during the same stages A, B and C were 1299.18 +/- 374.40, 1873.64 +/- 276.26 and 1669.36 +/- 537.92 dynes sec cm(-5) respectively, with significant high mean values at stages B and C compared to mean value at stage A (P<0.05). CONCLUSIONS: Morbid obesity and pneumoperitoneum have significant effects on hemodynamics. However, it appears that these changes were of marginal clinical significance.