Nodular lymphocyte-predominant Hodgkin lymphoma characteristics, management of primary and relapsed/refractory disease and outcome analysis: the first comprehensive report from the Middle East.

BACKGROUND: Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is an uncommon variant of Hodgkin lymphoma. There is limited data on treatment, management of refractory and relapsed disease, and long-term outcome. Many registries or country-wide data reports are unable to provide detailed primary and subsequent management. We are reporting our observation on patient's characteristics, management, and outcome. METHODS: This single-institution retrospective cohort analysis includes NLPHL patients seen from 1998 to July 2019. We used Fisher's exact test, chi-square, and Kaplan-Meier (KM) method for various analyses. RESULTS: Two hundred patients were identified, (6.34% of all the HL). Male:female was 3:1. The median age at diagnosis was 22 years (4-79 years). Stage I-II in 145 (72.5%) cases. One hundred patients (50%) received chemotherapy, 68 (34%) chemotherapy + radiation therapy (RT); 87% of all chemotherapy was ABVD (adriamycin, bleomycin, vinblastine, dacarbazine). Thirteen patients (6.5%) received RT alone and 16 (8%) had surgery alone. Complete response in 82%, partial response in 5.5% and progressive disease in 10.5%. The median follow is 60 months (5-246). Median 5 and 10 years overall survival (OS) is 94.8 and 92.4% (stages I-II, 97.7 and 97.7%, stage III-IV, 94.8 and 92.4%). Median event-free survival (EFS) is 62.3 and 54% respectively (stage I-II, 72 and 64%, stage III-IV, 36.4 and 18.2%). Stage I-II vs III-IV OS (p = < 0.001) and EFS (p = < 0.001) were significant. For stage I-II, 5 year EFS of chemotherapy + RT (83.3%) was superior to chemotherapy alone (60%, p = 0.008). Five year EFS for early favorable (80%), early unfavorable (60%), and advanced (36.4%) was significant (p = < 0.001). Eleven patients (5.5%) had high-grade transformation. Twenty-nine patients underwent HDC auto-SCT, all are alive (28 in remission). 25% of patients had pathologically proved nodal hyperplasia at some point in time. CONCLUSION: OS of NLPHL is excellent and independent of treatment type. EFS is better for chemotherapy + RT than chemotherapy alone. Stem cell transplant in refractory / multiple relapses resulted in excellent disease control. There is a need to identify optimal treatment strategies accordingly to the risk stratification.

Effect of panretinal photocoagulation on optic nerve head blood flow with OCT angiography in patients with diabetic retinopathy.

OBJECTIVE: To investigate the effect of pan retinal photocoagulation (PRP) on Optic nerve head blood flow with OCT angiography in patients with proliferative diabetic retinopathy. METHODS: This prospective interventional study was conducted at Eye Unit III, Institute of Ophthalmology, Mayo Hospital, Lahore, over a period of seven months i.e. from 10th May 2019 to 10th November 2019. Thirty-five patients having proliferative diabetic retinopathy were included in this study. Ocular blood flow was measured with OCT Angiography, then pan retinal photocoagulation was performed and patients were called for follow-up after one month and ocular blood flow was measured again with OCT Angiography. The difference in the blood flow was calculated. Frequencies and percentages were calculated for the categorical data and mean and standard deviations were calculated for the continuous data. Wilcoxon matched-pairs signed rank test was performed and effects of PRP on blood flow were compared. Significance level was taken as p≤0.05. RESULTS: Out of 35 patients, 30 patients (85.71%) had decreased post PRP blood flow, four patients (11.43%) had increased post PRP blood flow and one patient (2.86%) did not have any effect. CONCLUSION: OCT angiography revealed there is significantly reduced optic nerve head blood flow in PRP treated eyes compared to non-PRP treated eyes.

CHARACTERISTICS AND OUTCOMES OF PATIENTS WITH CHRONIC MYELOGENOUS LEUKEMIA IN BLAST CRISIS.

BACKGROUND: Chronic myelogenous leukaemia (CML) is a hematopoietic stem cell disease with a relatively stable clinical course. Survival has increased with addition of Tyrosine Kinase inhibitors (TKI's). Its conversion into blast crises (BC) heralds an accelerated clinical course that is less responsive to treatment and has high mortality. METHODS: Clinical records of 20 patients with CML who transformed to BC in two years between January 2012 and December 2013 were reviewed. RESULTS: Out of total 240 patients, 20 (8.3%) transformed to blast crisis; among them 75% were males and 25% females. Mean age was 37.9 years (24-58 years) and 19 patients were positive for t (9; 22) (q34; q11) translocation at the time of transformation. The mean initial blood cell count was 204 (range: 33 to 526). Imatinib was offered in 76% of patients. The average duration between diagnosis and transformation to blast crises was 201 days (range: 24-333 days). Eight patients (40%) were transformed to acute myeloid leukaemia (AML) and 12 (60%) had acute lymphoblastic leukaemia (ALL). These patients were treated with standard AML/ALL type induction chemotherapy except one who died early. During the study period, 11 patients died. Median survival for whole group was 55 days. On bivariate and multivariate linear regression analyses mortality was not, significantly associated with the duration between diagnosis and development of blast crises or the type of treatment received. CONCLUSION: Treatment of BC remains a challenge, particularly in under resourced areas where allogeneic hematopoietic stem cell transplantation (Allo-SCT) facility is sparse. Outcomes remain dismal in majority of these patients.

Outcomes among patients with sex cord stromal tumour of ovary: experience from Pakistan.

BACKGROUND: Ovarian sex cord-stromal tumours (SCST) are relatively uncommon neoplasms that account for approximately 5-7% of all primary ovarian tumours. The aim was to report experience with sex cord stromal tumours (SCST) of ovary in a low and middle income country. METHODS: Clinical records of 56 patients with histopathologically-established SCST of ovary admitted to a tertiary care cancer hospital in Pakistan between April 1995 and December 2011 were reviewed. RESULTS: Median age at presentation was 41 years (Range 4-77). Forty one (73%) patients were premenopausal and 15 (26.8%) were postmenopausal. The most common presenting complaint was abdominal pain (28.1%). Thirty seven patients (66%) had stage-I, 2 had stage II and II each, and 15 (26.8%) had stage IV disease. Five years survival in patients with early stage (stages I & II) was 91% while in those in the late stage (II & IV) was 84% (p=0.79). Histopathologically, 49 patients (85.7%) had Granulosa cell tumour, and 7 (12.5%) had Sertoli Lyedig cell tumour. CA-125 was high only in 8 patients (14.3%). Adjuvant chemotherapy was give in 16 (28.6%). Thirty six (64%) were disease free at last follow up, 10 (18%) succumbed to disease and 10 (18%) were alive with disease. On univariate and multivariate analyses, late stage at presentation was the sole factor significantly associated with mortality. CONCLUSION: Ovarian sex cord-stromal tumours of ovary are relatively uncommon malignancies with good prognosis if diagnosed early and treated adequately. Survival in our study was comparable to those reported elsewhere. Among various factors, late stage of tumour at presentation was found to be the only factor significantly associated with mortality.

Piperacillin-tazobactam as a cost effective monotherapy in febrile neutropenia.

BACKGROUND: Fever in neutropenic patient is a medical emergency. Timely intervention with antibiotics has been demonstrated to be effective. We assessed Piperacillin-Tazobactem as a cost effective mono-therapy in solid malignancy patients in our institution in relation to dual antibiotic therapy and other monotherapies. METHODS: This study was conducted to determine the efficacy, and cost effectiveness of Piperacillin-Tazobactem as monotherapy in febrile neutropenia. Total 150 patients with chemotherapy induced febrile neutropenia were selected. Piperacillin-Tazobactem was given intravenously 4500 mg every 6 hour. Outcome was assessed as success and failure. Success was defined as afebrile for four consecutive days, clearance of signs of infection, no new cultures, and no recurrence of primary infection after completion of therapy. Failure was defined as modification or addition of antibiotic due to clinical deterioration, cultured organism resistant to Piperacillin-Tazobactem and Death. RESULTS: The mean age was 43 years, 31% males and 69% were females. Out of total 150 patients, 73 patients were of breast carcinoma. There were 143 patients with negative blood cultures, and 7 patients with positive blood cultures, out of which 3 patients were resistant to Piperacillin-Tazobactem. Success was achieved in 83.3% of total patients. Daily cost of Piperacillin-Tazobactem was much less in relation to other monotherapies and dual antibiotic therapy including Gentamicin. None of the patient had adverse effects of Piperacillin-Tazobactem. CONCLUSION: We concluded that Piperacillin-Tazobactem is a safe, well tolerated as well as cost effective monotherapy in patient with febrile neutropenia with solid cancers. Only two percent organisms were resistant to Pipercillin-Tazobactam.