Background Romiplostim, a thrombopoietin (TPO) receptor antagonist, promotes tri-lineage hematopoiesis in patients with acquired aplastic anemia (AA). However, its efficacy as a first-line treatment ...in combination with an immunosuppressant, i.e., anti-thymocyte globulin (ATG) and cyclosporine (CSA), remains unexplored. Objective To assess the efficacy and safety of romiplostim in combination with ATG and CSA as first-line treatment in patients with AA. Method A single-center, retrospective study of AA patients, where data of patients administered with ATG + CSA + romiplostim as a first-line treatment was included. Romiplostim 5 µg/kg weekly for one month; post that, the dose was increased to 10 µg/kg weekly for the next five months. The primary outcome involves the overall response rate and hematological response at baseline, three months, and six months. Result Data from 12 patients with a median age of 18 years was evaluated. At a median follow-up of six months, 25% achieved a complete response, 41.6% achieved a partial response, and 16.7% had no response. Improvement in tri-lineage hematopoietic response had been seen at six months from baseline, with improvement in absolute neutrophil count (ANC) and platelet count (PC) being the most significant, with an increase of >100% from baseline, followed by total leukocyte count (TLC) (75.13%) and hemoglobin (Hb) (66.07%) from baseline. Two deaths were reported during the treatment. Conclusion Romiplostim, in combination with ATG plus CSA, demonstrated clinically significant outcomes as a first-line treatment in patients with AA. Further studies are required to confirm these findings in larger populations to assess long-term outcomes.
An amine-decorated zirconium based metal organic framework (MOF) UiO-66-NH
2
with rod shape morphology was synthesized by solvothermal process using 2-aminoterephthalic acid as an organic linker. ...Crystallinity of synthesized MOF material was confirmed with PXRD technique. MOF was employed as selective and sensitive sensor for ultra-trace detection of 2,4,6-trinitrophenol (TNP) in aqueous matrix, even in coexistence with other competitive nitroaromatic analytes. High value of Stern-Volmer quenching constant K
sv
(1.106 × 10
5
M
− 1
), plausible photoluminescent quenching efficiency (97.8%) and lower detection limit (0.95 µM/217ng mL
− 1
) ascertained extraordinary sensitivity of developed MOF for TNP. Density functional theory calculations and electrostatic interactions (i.e. ionic interaction, H-bonding and π-π interaction) indicated that electron and energy transfer processes play a key role in turn-off quenching response of UiO-66-NH
2
sensor. Spiked real samples were analysed to validate the developed method, which satisfactorily established the developed MOF sensor as an efficient tool for analysis.
Graphical Abstract
Highlights
An amine-decorated zirconium based metal organic framework (UiO-66-NH
2
) was synthesised.
Developed sensor detected 2,4,6-trinitrophenol (TNP) in water samples.
Detection of TNP was done in presence of other potentially competitive nitroaromatic analytes.
Lower detection limit (0.95 µM/217ng mL
− 1
) ascertained extraordinary sensitivity of developed UiO-66-NH
2
for TNP.
Electron and energy transfer processes justify the sensitivity of UiO-66-NH
2
sensor for TNP.
Thrombotic thrombocytopenic purpura (TTP) which can cause significant mortality is a thrombotic microangiopathy due to deficiency of VWF cleaving protease ADAMTS13 and as per medical literature there ...are examples that TTP can be caused by COVID 19 infection. A 35 years old female after admission with right sided weakness and slurring of speech was found to be COVID positive and diagnosed as a case of TTP. Patient had absent ADAMTS13 level on day 1. Treatment was started with therapeutic plasma exchange (TPE) later injection Vincristine and Rituximab was given after 4th TPE as it was suspected as refractory case. Finally patient received 16 TPE procedures with cryo poor plasma as exchange fluid and gradually her platelet count started to maintain normal and she was discharged. Specific management and such association of this type of cases need to be studied more judiciously.
Burkitt's Lymphoma (BL) can produce various clinical presentations and imaging appearances, according to the predilection of the different subtypes for certain sites. The gold standard treatment for ...BL remains the Magrath protocol as modified by the UK Lymphoma Group, as Cyclophosphamide, vincristine, doxorubicin, high-dose methotrexate (CODOX-M)/ifosfamide, etoposide, and high-dose cytarabine (IVAC) is an alternating non-cross-resistant regimen. Rituximab is now routinely used in BL treatment based on its strong CD20 expression.
We present a case of an 11-year-old female with Burkitt Lymphoma in the Pelvis. The patient was managed conservatively by 4 cycles of R-CODOX-M/R-IVAC for complete recovery of a pelvic lytic lesion. After the completion of chemotherapy, the patient got drastic clinical improvement with a massive decrease in the size of the swelling.
To conclude Pelvic Lytic lesion with biopsy and IHC proven Burkitt Lymphoma, can be managed conservatively without surgery with adequate histology-based chemotherapy regimen.
Level V.
Visceral leishmaniasis (VL), also known as kala-azar (black fever in Hindi), is a disease primarily caused by Leishmania donovani. The most important clinical manifestation of visceral leishmaniasis ...is fever. Nonspecific laboratory findings of visceral leishmaniasis include anemia, neutropenia, eosinopenia, and thrombocytopenia. Definitive diagnosis of visceral leishmaniasis requires the demonstration of either parasite by smear or tissue by culture (usually bone marrow or spleen). Myasthenia gravis is an autoimmune disease caused by antibodies to acetylcholine receptors in the post-junctional membrane of the neuromuscular junction. It typically presents with fatigable muscle weakness without any sensory or brain involvement. It is usually treated with corticosteroids and immunosuppressants like azathioprine. Here we encountered a confirmed case of myasthenia gravis on azathioprine with pancytopenia. While working up to evaluate pancytopenia, bone marrow examination revealed presence of Donovan bodies and the patient showed good response to liposomal amphotericin-B. In retrospect, a case of myasthenia gravis, who presented with pancytopenia presumably drug-induced, was found to have visceral leishmaniasis.
Cold agglutinin disease, commonly affecting females in the their seventh decade of life, is otherwise a rare entity and has an incidence of one case per million people per year. Cold reactive ...antibody is associated with autoimmune haemolytic anaemia (AIHA) in approximately 20% of the cases. Although the occurrence of AIHA in patients with non-Hodgkin's lymphomas is well known, aggressive lymphomas such as diffuse large B cell lymphoma (DLBCL) initially presenting as cold agglutinin AIHA is extremely rare. Here, we describe a case of DLBCL presenting few months before the diagnosis as cold antibody-AIHA.xs