A Bi-Lineal Acute Leukemia (T/Myeloid, NOS) with Complex Cytogenetic Abnormalities

We report an unusual case of bi-lineal mixed-phenotype acute leukemia (T/Myeloid, NOS) with complex cytogenetic abnormalities in a 2-year-old boy. Despite attaining complete remission with therapy, he succumbed to status epilepticus following febrile illness. Flow cytometry represents the current standard of care for the diagnosis of this malignancy and the approach adopted in our case is discussed.     

Parvovirus B-19 induced acute pure red cell aplasia in patients with chronic lymphocytic leukemia and neurofibromatosis type-1

Parvovirus B19 induced pure red cell aplasia (PRCA) has been previously reported in a variety of settings. We present two cases, an adult patient with chronic lymphocytic leukemia (CLL) and a child with neurofibromatosis type-1 (NF-1), where the abrupt appearance of severe anemia raised ominous clinical suspicions. Evidence of recent parvovirus B19 infection in association with the selective erythroid precursor deficiency in marrow helped exclude other etiologies.

We emphasize the importance of bearing this infectious agent in mind, even when there are associated disorders (such as CLL) that may independently cause PRCA. An association of NF-1 with acute PRCA has not been described in indexed English literature in the past.     

“Through the looking glass: 10-year-single centre experience in cardiac electrophysiology and radiofrequency ablation”

Background

Radiofrequency catheter ablation is an emerging curative modality of treatment for several types of cardiac arrhythmias. The aim of our study was to evaluate our experience of electrophysiology procedures over a 10-year-period and compare it with the published literature.

Methods and result

All patients undergoing cardiac electrophysiology and radiofrequency ablation procedures during the period from 01 January 2003 to 30 April 2012 were included in this study. The study analyzed 892 patients of which atrioventricular nodal reentrant tachycardia (AVNRT) was n = 513 (57.5%), accessory pathways n = 230 (25.8%), atrial tachycardia n = 26 (2.9%), atrial flutter n = 15 (1.7%), atrial fibrillation = 9 (1.0%), ventricular tachycardia n = 37 (4.2%) and diagnostic studies n = 62 (6.9%). Three-dimensional electro-anatomic mapping was performed in n = 17 (1.9%). The success rate was 99% with AVNRT, 94% with accessory pathway, 81% with atrial tachycardia, 64% with ventricular tachycardia and 34% with atrial fibrillation. The major and serious complications rate was 0.45%.

Conclusions

The procedural success rate and the complication rate in this real-world study of the Armed Forces were comparable/superior to the reported literature.     

Combination therapy of Tocilizumab and steroid for management of COVID-19 associated cytokine release syndrome: A single center experience from Pune,Western India

Cytokine release syndrome (CRS) or cytokine storm is thought to be the cause of inflammatory lung damage, worsening pneumonia and death in patients with COVID-19. Steroids (Methylprednislone or Dexamethasone) and Tocilizumab (TCZ), an interleukin-6 receptor antagonist, are approved for treatment of CRS in India. The aim of this study was to evaluate the efficacy and safety of combination therapy of TCZ and steroid in COVID-19 associated CRS.This retrospective cohort study was conducted at Noble hospital and Research Centre (NHRC), Pune, India between April 2 and November 2, 2020. All patients administered TCZ and steroids during this period were included. The primary endpoint was incidence of all cause mortality. Secondary outcomes studied were need for mechanical ventilation and incidence of systemic and infectious complications. Baseline and time dependent risk factors significantly associated with death were identified by Relative risk estimation.Out of 2831 admitted patients, 515 (24.3% females) were administered TCZ and steroids. There were 135 deaths (26.2%), while 380 patients (73.8%) had clinical improvement. Mechanical ventilation was required in 242 (47%) patients. Of these, 44.2% (107/242) recovered and were weaned off the ventilator. Thirty seven percent patients were managed in wards and did not need intensive care unit (ICU) admission. Infectious complications like hospital acquired pneumonia, blood stream bacterial and fungal infections were observed in 2.13%, 2.13% and 0.06% patients respectively. Age ≥ 60 years (P = .014), presence of co-morbidities like hypertension (P = .011), IL-6 ≥ 100 pg/ml (P = .002), D-dimer ≥ 1000 ng/ml (P < .0001), CT severity index ≥ 18 (P < .0001) and systemic complications like lung fibrosis (P = .019), cardiac arrhythmia (P < .0001), hypotension (P < .0001) and encephalopathy (P < .0001) were associated with increased risk of death.Combination therapy of TCZ and steroids is likely to be safe and effective in management of COVID-19 associated cytokine release syndrome. Efficacy of this anti-inflammatory combination therapy needs to be validated in randomized controlled trials.     

Glycemic Properties of Trichosanthes dioica Leaves

In continuation of our previous work on Trichosanthes dioica Roxb. (Cucurbitaceae), the aim of this study was to screen the glycemic attributes of an aqueous extract of Trichosanthes dioica leaves in normal as well as various diabetic models. The variable doses of 250, 500, and 750 mg kg^{? 1} body weight (bw) of the extract were administered orally to normal and streptozotocin (STZ)-induced sub- and mild-diabetic rats in order to define its glycemic potential. The dose of 500 mg kg^{? 1} bw was identified as the most effective dose which brings down the blood glucose level (BGL) by 32.9% (P < 0.001) at 6 h during fasting blood glucose (FBG) studies in normal rats. However, glucose tolerance test (GTT) showed the maximum reduction of 30.9% (P < 0.001) in BGL at 5 h in normal rats with the same dose, whereas the reduction observed was by 40.3% and 88.6% (P < 0.001) in sub- and mild-diabetic rats, respectively, at 3 h of glucose administration only. This evidence clearly indicates that the aqueous extract of Trichosanthes dioica leaves has good hypoglycemic potential along with a high anti-diabetic profile.     

Posterior capsule dehiscence during phacoemulsification and manual extracapsular cataract extraction: comparison of outcomes

PURPOSE: To compare the intraoperative behavior and postoperative outcomes of posterior capsule dehiscence during phacoemulsification and during manual extracapsular cataract extraction (ECCE). SETTING: L.V. Prasad Eye Institute, Hyderabad, India. METHODS: This study was a retrospective chart review of consecutive cases of posterior capsule dehiscence over 2 years in patients having phacoemulsification or manual ECCE. A chi-square test was used for statistical comparison of the results in the 2 groups. RESULTS: In the 127 eyes with posterior capsule dehiscence, the incidence of vitreous prolapse was comparable between the 2 groups (phacoemulsification, 61.6%; ECCE, 62.7%). In the phacoemulsification group, vitreous prolapse occurred more frequently if nuclear fragments were present at the time of posterior capsule dehiscence than if they were present during cortex removal (P =.05). Posterior dislocation of nuclear fragments occurred in 4 eyes in the phacoemulsification group. Increased anterior uveitis in the early postoperative period occurred more frequently in the ECCE group (P =.02). The visual outcomes were similar between the 2 groups. CONCLUSIONS: Even though differences existed between the intraoperative factors influencing the management of posterior capsule dehiscence during phacoemulsification and ECCE, the final anatomic and visual outcomes were comparable.     

ABCA12 homozygous mutation in harlequin ichthyosis: Survival without systemic retinoids

Harlequin ichthyosis (HI) is associated with high mortality. Early systemic retinoids are widely used, although their use remains debatable. We reported two neonates with homozygous mutations in ABCA12 consistent with harlequin ichthyosis who survived to discharge home with intensive care and without use of systemic retinoids.