Isolated palatal weakness without optic neuritis as the presenting manifestation of multiple sclerosis and its diagnostic dilemma with acute disseminated encephalomyelitis in a young boy

We present a case of a young boy who at initial presentation was diagnosed as acute disseminated encephalomyelitis (ADEM) but subsequently on follow-up was diagnosed as multiple sclerosis (MS). Differentiating ADEM from MS in their first presentation can be tricky as the features may not be typical of anyone. The importance lies in the close follow-up of these patients

Bilateral painful parotid lumps and a lump in the groin: An uncommon presentation of common Kikuchi's disease

Kikuchi-Fujimoto disease (KFD) is an under-recognized disease most commonly presenting with cervical lymphadenopathy, fever, and cytopenias in young females. Bilateral parotid enlargement is usually caused by infections (e.g., mumps) and autoimmune conditions (e.g., Sjogren syndrome). Parotid enlargement, inguinal lymphadenopathy, and pyrexia of unknown origin are uncommon presenting features of KFD and should be suspected in the appropriate setting

Refractory anemia in human immunodeficiency virus: Expect the unexpected

Pure red cell aplasia (PRCA) is an uncommon hematological disorder affecting selectively the erythroid cell lines. PRCA is defined as anemia with normal leukocyte and platelet counts, a corrected reticulocyte count <1%, <5% erythroid precursors in the bone marrow and an absence of hemolysis. We describe a case of Zidovudine (AZT) induced PRCA causing severe anemia in a patient taking antiretroviral therapy (ART) after 4 months of starting therapy and in whom all other causes were excluded. The hematological abnormalities resolved after AZT was replaced with tenofovir and the patient remained transfusion independent thereafter. A slowly progressive normocytic-normochromic anemia and reticulocytopenia, without leukopenia and thrombocytopenia in a patient, should raise the suspicion of PRCA. Search for underlying diseases, infections and drugs may help in the diagnosis and etiology of acquired PRCA. Elimination of potentially causative factors may induce complete recovery. AZT is a well-known cause of anemia and thus should be used with caution in the initiation of ART

Study of clinical characteristics, risk factors and outcomes for tuberculosis post allogeneic stem cell transplant: never count it out

Background: Allogeneic stem cell transplant (AlloSCT) recipients remain at a higher risk of developing tuberculosis (TB), especially in endemic populations. We conducted a retrospective study to identify the incidence, clinical presentation, and risk factors for active TB among our alloSCT recipients. Methods: Records of all patients transplanted between 1 January 2012 and 31 July 2020 were reviewed. Patients were followed up for outcome until 30 September 2020. None of the patients received prophylactic anti-tubercular drugs. Proven diagnosis of active TB was considered if Mycobacterium tuberculosis (MTB) was cultured from clinical samples or acid-fast bacilli (AFB) or MTB demonstrated on Ziehl-Neelsen (ZN) staining or histopathology or XPERT MTB, while probable diagnosis of TB was considered if histopathology findings were suggestive of caseation necrosis/epithelioid cell granulomas without any evidence of malignancy or lymphocyte rich exudative effusions (pleural/pericardial) without an alternative cause. Results: Among 381 alloSCT recipients, 15 patients (3.9%) developed TB at median of 246 (74–279) days post AlloSCT, after being symptomatic for a median of 22 (7–60) days, amounting to a cumulative incidence of 4.9%. All patients were started on four-drug anti tubercular therapy, ATT [Rifampicin, Isoniazid, Ethambutol, Pyrazinamide (RHEZ)], of which five patients developed hepatotoxicity at a median of 12 days after start of ATT, leading to drug modification. At last follow up, TB was cured in 13 (86.67%) patients, one succumbed to disease relapse, while others are still on treatment. Age ⩾ 30 years, immunosuppression for graft versus host disease (GvHD) > 6 months, prior use of tyrosine kinase inhibitors (TKI) and chronic GvHD on univariate analysis and immunosuppression for GvHD > 6 months on multivariate analysis were found to be associated with development of TB. Conclusion: A high index of suspicion with timely workup and treatment of TB is the key in AlloSCT recipients, especially in endemic TB populations

Extract Mitigates Experimental Acute Graft versus Host Disease Without Abrogating Graft Versus Leukemia Effect

Acute graft versus host disease (aGvHD) is the major contributor of nonrelapse mortality in alloHSCT. It is associated with an inflammatory immune response manifesting as cytokine storm with ensuing damage to target organs such as liver, gut, and skin. Prevention of aGvHD while retaining the beneficial graft versus leukemia (GvL) effect remains a major challenge. Withania somnifera extract (WSE) is known for its anti-inflammatory, immune-modulatory, and anticancer properties, which are appealing in the context of aGvHD. Herein, we demonstrated that prophylactic and therapeutic use of WSE in experimental model of alloHSCT mitigates aGvHD-associated morbidity and mortality. In the prophylaxis study, a dose of 75 mg/kg of WSE offered greatest protection against death due to aGvHD (hazard ratio [HR] = 0.15 [0.03-0.68], P ≤ .01), whereas 250 mg/kg was most effective for the treatment of aGvHD (HR = 0.16 [0.05-0.5], P ≤ .01). WSE treatment protected liver, gut, and skin from damage by inhibiting cytokine storm and lymphocytic infiltration to aGvHD target organs. In addition, WSE did not compromise the GvL effect, as alloHSCT with or without WSE did not allow the leukemic A20 cells to grow. In fact, WSE showed marginal antileukemic effect in vivo . WSE is currently under clinical investigation for the prevention and treatment of aGvHD

sj-docx-1-cll-10.1177_09636897241226573 – Supplemental material for Withania Somnifera Extract Mitigates Experimental Acute Graft versus Host Disease Without Abrogating Graft Versus Leukemia Effect

Supplemental material, sj-docx-1-cll-10.1177_09636897241226573 for Withania Somnifera Extract Mitigates Experimental Acute Graft versus Host Disease Without Abrogating Graft Versus Leukemia Effect by Saurabh Kumar Gupta, Dievya Gohil, Mohd Bashar Momin, Subhash Yadav, Akanksha Chichra, Sachin Punatar, Anant Gokarn, Sumeet Mirgh, Nishant Jindal, Lingaraj Nayak, Lal Hingorani, Navin Khattry and Vikram Gota in Cell Transplantation</p