Outcomes of Tyrosine Kinase Inhibitors Maintenance Therapy with or without Allogeneic Hematopoietic Stem Cell Transplantation in Philadelphia Chromosome Positive Acute Lymphoblastic Leukemia in First Complete Remission: A Systematic Review and Meta-Analysis.

We conducted a systematic review and meta-analysis to compare outcomes of tyrosine kinase inhibitor (TKI) maintenance therapy with or without allogeneic hematopoietic stem cell transplantation (HSCT) in Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL) in first remission (CR1). A literature search was performed on PubMed, Cochrane, and Clinical trials.gov. After screening 1720 articles, 12 studies were included. Proportions and odds ratios (OR) with 95% confidence intervals (CI) were computed. I2 provides an estimate of the percentage of variability in results across studies that is due to real differences and not due to chance. Of 1039 patients, 635 (61%) had TKI alone and 404 (39%) patients had HSCT followed by TKI. At 3 years, a trend towards poor overall survival (OS; OR 0.67, 95% CI 0.39-1.15, I2 = 68%), (disease-free survival; OR 0.58, 95% CI 0.26-1.29, I2 = 76%), and higher relapse rate (RR; OR = 2.52, 95% CI = 1.66-3.83, I2 = 26%) was seen with TKI alone compared to HSCT-TKI. Although HSCT followed by TKI maintenance in Ph+ ALL has long been considered standard of care, the introduction of potent third-generation TKIs and bispecific T-cell engagers such as Blinatumomab has significantly improved outcomes while sparing the need for HSCT in newly diagnosed patients.

A Case Report on Neuroleptic Malignant Syndrome (NMS): How to Approach an Early Diagnosis.

Neuroleptic malignant syndrome (NMS) is a life-threatening neurological emergency that has been observed to occur in some patients following the administration of anti-dopaminergic agents or the rapid withdrawal of dopaminergic medications. In this report, the authors present a case of a 51-year-old male patient with a known history of cocaine abuse, who was given quetiapine during his hospitalization. This precipitated an episode of NMS that eventually concluded uneventfully due to quick diagnosis and management. Prompt recognition of the condition is required to reduce significant morbidity and mortality. Ultimately, maintaining vigilance for the clinical features of NMS is crucial for timely diagnosis and intervention.

Superior mesenteric artery syndrome in a malnourished female: A rare cause of abdominal pain.

Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction that can go undiagnosed, exacerbating weight loss in an already significantly malnourished patient. Diagnosis is often challenging, however, can be made by keeping a high index of suspicion based on the clinical presentation. The pathology involves a reduction in the amount of fat pad between the abdominal aorta and superior mesenteric artery (SMA) leading to a reduction of aorto-mesenteric angle and consequent compression of mostly third part of the duodenum. Management is usually conservative, however, if conservative treatment fails, surgical intervention is warranted. Our patient was a 20-year-old female who presented to us with nausea, vomiting, weight loss, and abdominal pain. The presence of obstructive symptoms along with imaging (CT scan) lead to the diagnosis of SMA syndrome and she improved with conservative management. Informed consent was obtained for this study.

A Remarkable Case of Acute Motor-Sensory Axonal Polyneuropathy (AMSAN) Variant of Guillain Barré Syndrome, in a Diabetic Patient Infected With COVID-19: A Case Report and Review of the Literature.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which causes coronavirus disease- 2019 (COVID-19), has been a global epidemic in our healthcare system. SARS-CoV-2 primarily affects the respiratory system, but neurological involvement has also been reported, including Guillain-Barré syndrome (GBS) development. Case Presentation: A 58-year-old male with known co-morbid hypertension and type 2 diabetes mellitus presented to the emergency room with complaints of worsening shortness of breath, dry cough, and fever for the past 10 days. On day 20 of hospitalization, he developed neurological symptoms after being tested positive for COVID-19. A neuroelectrophysiology study was conducted to evaluate neurological symptoms and suggested that the patient suffers from acute motor-sensory axonal polyneuropathy (AMSAN). CSF analysis showed elevated protein levels that confirmed the diagnosis of GBS. He was subsequently treated with oral prednisolone and IVIG, which improved neurological symptoms. Conclusion: Ever since the emergence of COVID-19, GBS has surfaced as to its potentially dangerous outcome. Healthcare professionals should be mindful of GBS and should rule it out in anyone having sensory symptoms or weakness during or after a COVID-19 infection. Its early detection and treatment can result in improved clinical outcomes.

Mitochondrial neurogastrointestinal encephalomyopathy in a Pakistani female: a case report.

BACKGROUND: Mitochondrial neurogastrointestinal encephalopathy is a rare multisystem autosomal recessive disease caused by mutations in the TYMP gene, that encodes for thymidine phosphorylase. Mitochondrial neurogastrointestinal encephalopathy is a progressive degenerative disease characterized by a distinctive tetrad of gastrointestinal dysmotility, peripheral neuropathy, ophthalmoplegia with ptosis, and asymptomatic leukoencephalopathy. It provides a diagnostic dilemma to physicians in regions like Pakistan because of a lack of genetic study availability and associated financial constraints of the population. However, with careful examination and a few basic investigations, mitochondrial neurogastrointestinal encephalopathy can be diagnosed by ruling out most of the close differentials. CASE PRESENTATION: We report the case of a 23-year-old Asian female whose chief complaints were epigastric pain, bilious emesis, weight loss for 3 months, and bilateral lower limb weakness for 20 days. All clinical signs and symptoms along with relevant investigations including nerve conduction studies, electromyography, and magnetic resonance imaging of the brain were highly suggestive of mitochondrial neurogastrointestinal encephalopathy syndrome. Because of financial constraints, genetic studies could not be performed. The patient was managed with a multidisciplinary approach involving gastroenterology, physiotherapy, and nutrition departments. Currently, therapeutic options for the disease include allogeneic hematopoietic stem cell transplant and carrier erythrocyte entrapped thymidine phosphorylase; however, these could not be provided to the patient owing to certain limitations. CONCLUSIONS: As misdiagnosis and delayed diagnosis are quite common in this disease, the prime objective of this case report is to increase the basic understanding of this disease, especially its signs and symptoms, and address the limitations regarding the diagnostic investigations and management of patients with mitochondrial neurogastrointestinal encephalopathy.

A Misdiagnosed Case of Schizoaffective Disorder With Bipolar Manifestations.

Bipolar and schizoaffective disorders are both psychiatric illnesses that share common traits, but also significant differences. Due to the close overlap in symptoms, obtaining the correct diagnosis can be difficult. The management of these patients often poses a challenge to clinicians. Five years ago, our patient was misdiagnosed with bipolar disorder with psychotic features. It was later discovered that she was suffering from schizoaffective disorder, bipolar type. The schizoaffective disorder involves symptoms of both schizophrenia and mood disorder.

Navigating a Curious Case of Unilateral Keratouveitis in Pakistan.

Keratouveitis is a rare but potentially dangerous condition of the eye that requires prompt management otherwise it may result in severe visual impairment. We report a challenging case of unilateral keratouveitis. A 40-year-old man presented with right eye erythema, epiphora, decreased vision, and severe pain from one month after failure to antibiotics treatment. Slit-lamp examination showed circumcorneal injection of conjunctiva, a central corneal ulcer, hypopyon and cells in the anterior chamber (AC). The culture and sensitivity of ocular tissue showed no growth of organisms. Intravitreal antibiotics (vancomycin and ceftazidime) along with anterior chamber tap led to resolution of the inflammation. The corneal ulcer was markedly reduced and vision was improved. This case emphasizes two important points. First, how to approach a case of keratouveitis of uncertain etiology, in a less resourceful situation. Second, how to treat unilateral keratouveitis with intravitreal antibiotics and anterior chamber/vitreous tap.