Short-course Venetoclax With Standard Chemotherapy Is Effective in Early T-cell Precursor Acute Lymphoblastic Leukemia.

BACKGROUND: Early T-cell precursor acute lymphoblastic leukemia (ETP ALL) is a high-risk subgroup of acute lymphoblastic leukemia characterized by unique immune phenotype and disease biology. ETP ALL cells share similarities with hematopoietic stem cells and myeloid progenitor cells. These patients have lower rates of complete remission and overall survival. High BCL2 expression is the main rationale for using venetoclax in ETP ALL. RESULTS: We report the treatment outcomes of 2 patients with ETP ALL who achieved minimal residual disease negative remission with the short course of venetoclax. CONCLUSIONS: Combination therapy of short-course venetoclax with Berlin-Frankfurt-Meunster 95 regimen is an effective regimen for treating patients with ETP ALL.

Daratumumab for pure red cell aplasia post ABO incompatible allogeneic hematopoietic stem cell transplant for aplastic anemia.

Pure red cell aplasia is a known complication after ABO incompatible stem cell transplant. Due to rarity of disease, no established treatment guidelines are available for PRCA. Daratumumab is a monoclonal antibody against CD38 expressed by plasma cells. In this report we present our experience of successfully managing a patient of post-transplant PRCA with daratumumab. Our patient had failed multiple lines of therapy prior to receiving daratumumab. Response was seen after the 3rd weekly dose of daratumumab.

Effectiveness of convalescent plasma in Indian patients with COVID-19.

BACKGROUND: Convalescent plasma (CP) is being used as a treatment option in hospitalized patients with COVID-19. Till date, there is conflicting evidence on efficacy of CP in reducing COVID-19 related mortality. OBJECTIVE: To evaluate the effect of CP on 28-day mortality reduction in patients with COVID-19. METHODS: We did a multi-centre, retrospective case control observational study from 1st May 2020 to 31st August 2020. A total of 1079 adult patients with moderate and severe COVID-19 requiring oxygen, were reviewed. Of these, 694 patients were admitted to ICU. Out of these, 333 were given CP along with best supportive care and remaining 361 received best supportive care only. RESULTS: In the overall group of 1079 patients, mortality in plasma vs no plasma group was statistically not significant (22.4% vs 18.5%; p = 0.125; OR = 1.27, 95% CI: 0.94--1.72). However, in patients with COVID-19 admitted to ICU, mortality was significantly lower in plasma group (25.5% vs 33.2%; p = 0.026; OR = 0.69, 95%CI: 0.50-0.96). This benefit of reduced mortality was most seen in age group 60 to 74 years (26.7% vs 43.0%; p = 0.004; OR = 0.48, 95% CI: 0.29-0.80), driven mostly by females of this age group (23.1% vs 53.5%; p = 0.013; OR = 0.26, 95% CI: 0.09-0.78). Significant difference in mortality was observed in patients with one comorbidity (22.3% vs 36.5%; p = 0.004; OR = 0.50, 95% CI: 0.31-0.80). Moreover, patients on ventilator had significantly lower mortality in the plasma arm (37.2% vs 49.3%; p = 0.009; OR = 0.61, 95% CI: 0.42-0.89); particularly so for patients on invasive mechanical ventilation (63.9% vs 82.9%; p = 0.014; OR = 0.37, 95% CI: 0.16-0.83). CONCLUSION: The use of CP was associated with reduced mortality in COVID-19 elderly patients admitted in ICU, above 60 years of age, particularly females, those with comorbidities and especially those who required some form of ventilation.

Effect of age, comorbidity and remission status on outcome of COVID-19 in patients with hematological malignancies.

BACKGROUND: There is scarcity of data on outcome of COVID-19 in patients with hematological malignancies. Primary objective of study was to analyse the 14-day and 28-day mortality. Secondary objectives were to correlate age, comorbidities and remission status with outcome. METHODS: Retrospective multicentre observational study conducted in 11 centres across India. Total 130 patients with hematological malignancies and COVID-19 were enrolled. RESULTS: Fever and cough were commonest presentation. Eleven percent patients were incidentally detected. Median age of our cohort was 49.5 years. Most of our patients had a lymphoid malignancy (n = 91). One-half patients (52%) had mild infection, while moderate and severe infections contributed to one-fourth each. Sixty seven patients (52%) needed oxygen For treatment of COVID-19 infection, half(n = 66) received antivirals. Median time to RT-PCR COVID-19 negativity was 17 days (7-49 days). Nearly three-fourth (n = 95) of our patients were on anticancer treatment at time of infection, of which nearly two-third (n = 59;64%) had a delay in chemotherapy. Overall, 20% (n = 26) patients succumbed. 14-day survival and 28-day survival for whole cohort was 85.4% and 80%, respectively. One patient succumbed outside the study period on day 39. Importantly, death rate at 1 month was 50% and 60% in relapse/refractory and severe disease cohorts, respectively. Elderly patients(age ≥ 60) (p = 0.009), and severe COVID-19 infection (p = 0.000) had a poor 14-day survival. The 28-day survival was significantly better for patients in remission (p = 0.04), non-severe infection (p = 0.00), and age < 60 years (p = 0.05). CONCLUSIONS: Elderly patients with hematological malignancy and severe covid-19 have worst outcomes specially when disease is not in remission.

Convalescent plasma therapy for severe Covid-19 in patients with hematological malignancies.

BACKGROUND: Data on convalescent plasma therapy (CPT) in patients of hematological malignancies with severe Covid-19 is scarce. OBJECTIVE: To study 14-day mortality in patients who received CPT. PATIENTS & METHODS: Retrospective multicentre observational study conducted in 4 centres treating haematological malignancies across Delhi-national capital region. Total 33 haematological malignancies patients with severe Covid-19 who received CPT were analysed. RESULTS: The median age of the study cohort was 62 years (18-80 years). Twenty one percent patients had 1 comorbidity, 18 % had 2 comorbidities and 6% patients had 3 and 5 comorbidities each. Twenty four patients were on active therapy. Sixty nine percent of patients required ICU stay. Twenty five patients received plasma therapy within 7 days (early) of diagnosis of Covid-19 infection. Median day of plasma infusion from date of diagnosis of Covid-19 infection was 4 days (range: 2-25 days). Patient who had early initiation of plasma therapy had shorter duration of hospitalisation (12.7 vs 24.3 days, p = 0.000). Overall mortality in the cohort was 45.5%. There was no effect of disease status, active therapy, presence of comorbidity on mortality. There was no difference in the mortality in patients receiving early vs late initiation of plasma therapy or in patients receiving one versus two plasma therapy. CONCLUSIONS: We provide a large series of patients with hematological malignancies and role of CPT in this group.

Discontinuation of Imatinib in a Child With Chronic Myeloid Leukemia.

Chronic myeloid leukemia (CML) has different disease biology with a more aggressive clinical course in children. Achieving treatment-free remission is the ideal goal for the pediatric CML population to avoid long-term toxicities of tyrosine kinase inhibitors. Here, we present our experience of stopping Imatinib in a pediatric patient of CML who had excessive weight gain with Imatinib. He is currently maintaining treatment-free remission for 15 months after stopping therapy at the time of last follow-up. The patient also had normalization of body mass index with the stopping of Imatinib.

Hematogone Hyperplasia Masquerading as Acute Lymphoblastic Leukemia With Concurrent Hypercupremia.

Alterations in copper homeostasis is an uncommon cause for hematologic alterations frequently presenting with dysplastic features in the bone marrow. Most of these alterations have been documented in adult patients with copper deficiency. Rare cases show hematogone hyperplasia in these patients. Effects of mild copper excess have not been documented in literature. We are describing a pediatric patient who presented with pancytopenia associated with hypercupraemia (excess of copper). Bone marrow examination showed hematogone hyperplasia. Interestingly, correction of serum copper levels with zinc therapy lead to complete improvement in pancytopenia. Hematogones had also reduced in subsequent marrow biopsy after therapy.

Prevalence of Flu-like Symptoms and COVID-19 in Healthcare Workers from India.

BACKGROUND: The current COVID-19 pandemic is unprecedented. As the numbers expand exponentially, a paucity of data regarding health care workers (HCWs), who are at the forefront of this disaster, exists. Hence we decided to conduct a study amongst the HCWs to determine the prevalence and risk factor stratification. METHODS: This was an online questionnaire-based survey of healthcare workers conducted at Max Super Speciality Hospital, Saket, New Delhi, India from 23rd March to 30th April 2020. Data on flu-like symptoms, travel history, posting in high-risk or low risk zones, and prophylactic drugs was collected. RESULTS: Out of the 18000 HCWs who were approached 4403 responded and adequate data of 3667 was available for analysis. 14.7% had flu-like symptoms. 1.8% (20/1113) of the participants tested were positive for the virus. HCWs posted in the high-risk zones had more symptoms than those working in low-risk zones (169/539, 31.4% vs 679/3128, 21.7%), p<0.001; but no difference in COVID-19 positivity rates (p=0.849). Symptomatic HCWs had higher positivity (10/193, 5.2%) than the asymptomatic ones (10/920, 1.1%), p=0.001. HCQ was taken by 755/1113 (67.8%) people and 14 (1.9%) of these reported positive for the virus. CONCLUSION: This is the first study on healthcare workers from India to the best of our knowledge. Our findings suggest that posting in a high-risk zone with adequate PPE does not pose higher risk to the HCWs. Moreover, HCQ as a prophylactic has no use. CLINICALTRIALS.GOV IDENTIFIER: NCT04339608.

Increased complications and morbidity in children with hemophagocytic lymphohistiocytosis undergoing hematopoietic stem cell transplantation.

BACKGROUND: Hematopoietic stem cell transplantation (HSCT) is the only curative option for patients with primary hemophagocytic lymphohistiocytosis (HLH) and for patients with secondary HLH who fail to respond to therapy. METHODS: Retrospective study of HSCT for HLH with focus on complications and outcome. RESULTS: Eighteen children (10 males), with a median age of 1.2 yr (5 months-16 yr), received HSCT for HLH. Fourteen children had primary HLH. Four children underwent transplant while not in remission. Sixteen received myeloablative and two received reduced intensity conditioning regimen. A high incidence of complications was found: 13 (72%) children had 22 episodes of culture-proven infections; seven (38%) had hepatic veno-occlusive disease; nine (50%) developed respiratory complications; and nine (50%) required intensive care unit admission. Eight children had acute graft-versus-host disease (GVHD), and three developed chronic GVHD. Three patients died from multi-organ failure before day +100, and another patient died from pulmonary hemorrhage after day 100. Three patients failed to engraft (two developed recurrent HLH and died from complications after a second HSCT). Three of four children not in remission at the time of transplantation died. Actuarial survival at three yr was 61%. CONCLUSION: HSCT for HLH carries significant risks with high infection, organ dysfunction, and ICU admissions rates.

Allogeneic cord hematopoietic stem cell transplantation in an infant with primary myelofibrosis.

Primary myelofibrosis (PMF) is rare in children. An allogeneic hematopoietic stem cell transplantation (HSCT) is the only known curative therapy for severe cases. Here, we report the case of a female infant with PMF treated with allogeneic HSCT using an unrelated cord blood unit. She had successful reversal of her disease, but experienced complications related to transplant. This is the seventh reported case of HSCT for PMF in children, and the second using umbilical cord blood. We conclude that cord HSCT is a useful curative treatment option in children with PMF, but that efforts must be taken to reduce complications.

Thymic mucosa-associated lymphoid tissue lymphoma in an adolescent girl.

Mucosa-associated lymphoid tissue (MALT) lymphoma is very rare in children. We report the first case of pediatric thymic MALT lymphoma in an adolescent Asian girl. She presented with chest pain, dyspnea, and low-grade fever. A large anterior mediastinal mass was biopsied that confirmed the diagnosis of MALT lymphoma with trisomy 18. The patient had secondary immunodeficiency with low NK cell count and high IgA and IgG levels. Because of the advanced stage and the presence of trisomy 18, she was treated with cyclophosphamide, vincristine, prednisone, and rituximab, followed by involved-field radiotherapy. She is currently undergoing maintenance therapy with rituximab and remains in complete remission at 13 months from diagnosis. Thymic MALT lymphoma should be suspected in any Asian child with a cystic thymic mass and autoimmune disease or hyperglobinemia. Because of the slow proliferation rate of this type of lymphoma, a long-term follow-up is needed.

Outcomes of COVID-19 in Hematopoietic Stem Cell Transplant Recipients: Multicenter Retrospective Analysis.

COVID-19, caused by the severe acute respiratory syndrome corona virus 2 (SARS-CoV-2), was declared a pandemic by the World Health Organization on March 9, 2020. Hematopoietic stem-cell transplantation (HSCT) recipients may be highly susceptible to infection and related pulmonary complications due to nascent immune systems or organ damage from treatment-related toxicities. Poor outcomes in such group of patients were linked to older age, steroid therapy at the time of COVID-19 infection, and COVID-19 infection within a year of HSCT. We studied a cohort of 28 hematopoietic stem cell transplant recipients (male 17, M:F ratio of 1.5) with COVID-19 infection from 1st June 2020, through 31st December 2020 for outcome. Fever was the most common symptom at the time of presentation in 22 (78.5%) patients. Mortality rate at Day 28 and Day 42 was found to be 4/28 (14.3%) and 7/28 (25%) respectively. Patients within one year of HSCT and severe infection had higher day 28 mortality (with p values = 0.038)". There was no relation of mortality with type of transplant.