Significant reduction in morbidity and mortality have been documented in patients with sickle cell disease (HbSS) by most of the studies using hydroxyurea at a dose of 25-35 mg/kg/day or maximum tolerated dose. But toxicities, need for frequent monitoring, compliance and cost are important hurdles particularly in Indian set up. We undertook this study to find out the efficacy, safety compliance rate of low fixed dose of hydroxyurea (10 mg/kg/day) in patients presenting to our hospital and its impact on clinical profile and laboratory parameters. A cohort of 128 (82 males, 46 females) confirmed HbSS cases (each >18 years age, vaso-occlusive crisis >2/years and/ or rate of transfusion 1-2 units/month) with no disease related end organ damage were assessed prospectively between 2013 and 2016. They were started on 10 mg/kg/day hydroxyurea along with other supportive care and followed up monthly for 1 year. Clinical and laboratory parameters before and after therapy were reviewed and compared. In 92% of cases presenting with repeated vaso-occlusive crisis, VOC disappeared completely during follow up and in 8% we found significant reduction in severity as well as frequency of attacks ( < 0.01). Again in 87%, no further transfusion was required during follow up and in 13%, it further reduced the rate of transfusion ( < 0.01). The median time of response for VOC was 3 months and in transfusion requirement was 5 months. There was also significant reduction in S.Billirubin, S.LDH, disease related complications and rate of hospitalisation with significant improvement in Hb, MCV, and MCH. There is insignificant increase in HbF with median (1.5-2.4)% and in 5 cases >5%. We did not find any remarkable adverse effect of the drug during the study period. Low fixed dose hydroxyurea (10 mg/kg/day) is beneficial in reducing the vaso-occlusive crisis and transfusion requirement in adult HbSS Patients (Arab-Indian Haplotype). It is safe, suitable and is a effective mode of treatment in resource poor setting like India.
In COVID 19 pandemic, delivery and access of health care services have become challenging. Telemedicine services can be considered for management of patients with hematological diseases. This study included all patients who enrolled for telemedicine facility for hematology from May 15 to July 15, 2020. Patient's demographic and disease related parameters were recorded during the teleconsultation call. Overall satisfaction of attending doctor and patients were also recorded. A total of 1187 teleconsultation appointments were taken, of which 944 (79.6%) were successfully attended. Median age of patients was 38 years (range-0.5-78 years), with 38% females. 55% of successful calls were from patients suffering a malignant hematological disorder. 24% had an active complaint pertaining to their disease or treatment. Of these, 162 (17%) were asked for a physical consultation. A significant association was found between the requirement of physical consultation and diagnosis (p \ 0.001),
Introduction
To study the efficacy and safety of single large volume leukapheresis by using generic G-CSF or G-CSF plus Plerixafor in achieving adequate stem cell yield and various factors influencing thereof in newly diagnosed multiple myeloma patients undergoing autologous stem cell transplant .
Method
This prospective study was undertaken among 55 newly diagnosed multiple myeloma patients undergoing autologous stem cell transplant and aged between 18 and 75 years. Mobilization and harvesting of stem cells were performed by using GCSF or GCSF plus Plerixafor and large volume leukapheresis, respectively. A stem cell yield of ≥2 × 10
6
kg
–1
and the number of apheresis procedures were primary efficacy endpoints, while the ideal stem cells yield >5 × 10
6
kg
–1
, the engraftment day and D100 response/graft sustainability were secondary endpoints.
Result
The primary endpoint was achieved in all cases in both the groups by using a single LVL leukapheresis procedure. Fulfillment of all the secondary endpoints was satisfactory and comparable in both the groups. Age, pre-apheresis CD34+ count and number of interruptions during the LVL were significant factors influencing the stem cell yield (
p
< 0.05). Adverse drug reactions during the apheresis and post-ASCT period were manageable.
Conclusion
The LVL is safe and cost-effective in attaining a minimum of CD34+ cells in a single procedure with manageable adverse reactions. Judicious intervention during the procedure may be helpful in ensuring the adequate yield.
Classical CRAB features (hypercalcaemia, renal failure, anaemia, osteolytic lesions) have been traditionally defined in patients with plasma cell dyscrasia. But these can be rare and uncommon presentations of other chronic lymphoproliferative disorders (CLPD). The pathophysiological basis of CRAB features in other CLPD need to be explored further for better outcomes and therapeutic interventions. These can present a diagnostic dilemma and requires extensive workup to rule out coexisting malignancy and myeloma. Here, we report an unusual case of B CLPD in a middle-aged male who presented with classical CRAB features along with a brief literature review. After detailed investigations, he was diagnosed as chronic lymphocytic leukaemia, without any second malignancy and responded well to ibrutinib-based therapy.
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