Uncommon Presentation of Post-Transfusion Purpura in an Elderly Male: A Case Report and Unique Alloantibody Identification.

BACKGROUND Post-transfusion purpura (PTP) is a rare delayed adverse event characterized by severe thrombocytopenia associated with mucosal bleeding and purpura. PTP is associated with the development of alloantibodies to human platelet antigens (HPAs) and should be distinguished from other thrombocytopenic syndromes. This report is of a 69-year-old man with refractory cardiogenic shock and thrombocytopenia 4 days following blood transfusion, diagnosed with post-transfusion purpura. CASE REPORT A 69-year-old man was admitted to a tertiary medical center with refractory cardiogenic shock. Four days after he received 1 unit of packed red blood cells, his platelet count plummeted from 147 K/uL to <2 K/uL within hours, associated with delayed presentation of notable hematuria and femoral catheter oozing. An extensive thrombocytopenia work-up, including an initial platelet antibody screen, was unrevealing. The patient was treated with supportive transfusions, dexamethasone, and intravenous immunoglobulin, with rapid platelet recovery. Post-transfusion purpura panel testing later identified anti-human platelet antigen-5b antibodies, confirming the diagnosis. CONCLUSIONS This report presents an unusual course and presentation of post-transfusion purpura in an elderly man. Unusual features of this case include male sex, hyper-acuity of thrombocytopenia, lack of prior transfusions, exam findings, identification of a less common alloantibody, and negative initial platelet antigen screening. This report highlights the importance of monitoring patients for post-transfusion adverse events. Although PTP is rare, rapid diagnosis and management are required to control this potentially life-threatening condition.

Age-Specific Nomograms for Antral Follicle Count in Fertile and Infertile Indian Women: A Comparative Study.

Objectives The aim of this study was to develop age-specific nomograms for antral follicle count (AFC) in fertile and infertile Indian women and (2) to compare the influence of age on AFC in both groups. Setting and Design It is a prospective cross-sectional study in a tertiary-care hospital in north-central India. Methods and Material One-thousand four-hundred seventy-eight fertile and 1,447 infertile women (primary infertility) of reproductive age (18-49 years) were recruited. One-thousand one-hundred eighty-one fertile and 1,083 infertile women fulfilled the selection criteria for the study. Transvaginal ultrasonography was done on the second or third day of the menstrual cycle. Statistical Analysis Age-specific nomograms for AFC were built for the 3rd, 10th, 25th, 50th, 75th, 90th, and 97th percentiles in both groups. Correlation and regression analysis was done to estimate the relationship between the study variables. Statistical analysis was done by using IBM SPSS Statistics for Windows, version 20. Results At every age, each percentile value of AFC was lower in infertile than in fertile women. The decline of AFC with increasing age was linear in both fertile ( r = - 0.431, p < 0.001) and infertile ( r = - 0.520, p < 0.001) women; however, the rate was higher in the latter (0.50 follicle/year) than in former (0.44 follicle/year) group. The variation in AFC explained by age was 16.3% in fertile and 22.7% in infertile women. Conclusion AFC decreased linearly with advancing age in both fertile and infertile women, but more rapidly in the latter. The age only modestly explained the decline of AFC. The age-specific percentile thresholds for AFC should be used instead of age-independent constant thresholds in infertility counselling.

Diagnostic Role of Transvaginal Sonography and Magnetic Resonance Imaging in Adenomyosis of the Uterus and its Correlation with Histopathology.

Background and Objective: The prevalence of adenomyosis of the uterus varies from 5% to 70%, and there is no clear consensus on its imaging diagnostic criteria. The objective of this study was to evaluate the role of transvaginal sonography (TVS), combined TVS and color Doppler (TVS-CD), and magnetic resonance imaging (MRI) in the diagnosis of adenomyosis. Materials and Methods: This was a tertiary care hospital-based prospective study, in which 365 clinically suspected cases of adenomyosis were enrolled. All three types of imaging (TVS, TVS-CD, and MRI) were done in 233/365 patients, followed by hysterectomy in 50. Imaging features were correlated with the histopathological examination (HPE), which was taken as the gold standard for the diagnosis. The diagnostic performance of each imaging modality was assessed. Results: Among patients who underwent hysterectomy, 36/50 (72%) had adenomyosis on HPE, with or without associated benign gynecological abnormalities. Sensitivity, specificity, positive predictive value (PPV), negative PV (NPV), and diagnostic accuracy (DA) of MRI were higher than that of TVS-CD (91.67% vs. 77.78%, 85.71% vs. 78.57%, 94.29% vs. 90.32%, 80% vs. 57.89%, and 90% vs. 78%, respectively). TVS alone had lower diagnostic performance (specificity: 64.29%, PPV 84.85%, NPV 52.94%, and DA74%) than TVS-CD, but equal sensitivity (77.78%). Heterogeneous myometrium was the most sensitive (80.56%), while myometrial cyst was the most specific (92.86%) TVS feature. The maximum junctional zone thickness ≥12 mm was the most sensitive (97.22%), while the hyperintense myometrial focus was the most specific (100%) MRI feature. Conclusion: TVS-CD should be used as an initial diagnostic imaging modality in clinically suspected cases of adenomyosis; however, MRI due to better diagnostic efficacy should be the imaging modality of choice before subjecting such patients to hysterectomy.

Additional efficacy analysis of avatrombopag phase III data for the treatment of adults with immune thrombocytopenia.

Avatrombopag is an oral thrombopoietin receptor agonist (TPO-RA) that was approved in the US in 2019 for treatment of chronic immune thrombocytopenia (ITP). This post hoc analysis of the pivotal phase III study (NCT01438840) of avatrombopag in adult patients with ITP evaluated platelet count response to avatrombopag during the core study in different subgroups, and durability of response data in patients who responded to avatrombopag treatment both during the core phase (total population) and during the core and extension phase (total population and by subgroup). Loss of response (LOR [platelet count <30 × 109/L]) was defined as LOR over two consecutive scheduled visits. The response was generally similar between subgroups though a few differences were observed. The durability of response analysis showed that avatrombopag-treated patients maintained their response for 84.5% of time on treatment during the core phase and 83.3% during the core and extension phase; 55.2% of patients in the core phase and 52.3% in the core and extension phase never experienced LOR. We conclude that the initial response to avatrombopag is both stable and durable.

What is the context? Avatrombopag is a medicine that helps the body produce platelet cells, which are necessary for blood clotting.Avatrombopag is used to treat people with chronic immune thrombocytopenia (ITP); these patients have low numbers of platelet cells in their blood, so their blood may not clot efficiently, putting them at risk of uncontrolled bleeding.A phase III clinical study in patients with chronic ITP showed that platelet counts increased for most patients who were treated with avatrombopag; patients who had a platelet count ≥50 × 10⁹/L were considered to have a response to avatrombopag treatment.The present study analyzes data from a phase III clinical study to determine whether there are any characteristics that make a patient more or less likely to have a loss of response (LOR) while taking avatrombopag, whether the initial response to avatrombopag is stable, and how long the response lasts.For this analysis, LOR is defined as platelet count <30 × 10⁹/L for either four consecutive weeks or for two consecutive office visits while taking avatrombopag.What is new? In general, patient characteristics did not influence the likelihood of LOR.Patients who responded maintained their response for most of the time they were taking avatrombopag.Most patients did not experience LOR.What is the impact? The initial response to avatrombopag is stable and long-lasting in patients with chronic ITP.These findings indicate that patients with a variety of background characteristics can experience a durable platelet response with avatrombopag treatment.

Inverted-Y Ureteric Duplication With Paraureteric Diverticulum Presenting With Bladder Outlet Obstruction in an Infant- A Diagnostic Dilemma.

The ectopic ureter and paraureteric diverticulum are 2 known common urological anomalies of pediatric patients. Another rare entity is inverted-Y ureteric duplication. We report a case of a 3-month-old boy presented with bladder outlet obstruction, where surgical excision of large bladder diverticulum with left ureter and small kidney was done. Histopathology confirmed the presence of inverted-Y ureteric duplication with left dysplastic kidney. The report defines the first case of infantile bladder outlet obstruction having the co-existing congenital genitourinary anomaly of inverted Y-partial ureteric duplication with obstructive ectopic ureter and ipsilateral paraureteric diverticula.

Cryocrystalglobulinemia Leading to Multi-Organ Failure in Chronic Lymphocytic Leukemia Achieving Complete Renal Recovery.

Cryocrystalglobulinemia (CCG) is a rare and fatal subset of type I cryoglobulinemia that is classically associated with an underlying monoclonal gammopathy. Cryocrystalglobulins are created when immunoglobulins self-assemble into extracellular crystal arrays, which often leads to severe systemic hypoperfusion and occlusive vasculopathy that culminates in multi-organ failure. Most commonly, the resultant ischemia manifests as cutaneous lesions and renal insufficiency, which can progress to fulminant kidney failure requiring renal replacement therapy. CCG is commonly associated with lymphoproliferative disorders and is most frequently reported in the literature in context of plasma cell dyscrasias with minimal cases describing CCG secondary to other types of lymphoid neoplasms, especially those that attain complete organ recovery. We report a unique case of a patient who presented with multi-organ failure, including cryoglobulinemic glomerulonephritis (CryoGN) consistent with monoclonal gammopathy of renal significance (MGRS), who was found to have type I IgG kappa CCG due to chronic lymphocytic leukemia (CLL). With the assistance of plasmapheresis, hemodialysis, and clone-directed therapy, the patient achieved complete renal recovery. We highlight this uncommon entity to emphasize the clinical importance of early diagnosis and timely treatment given CCG's significant morbidity and mortality.

Facet Tropism/Inclination and Its Association with Intervertebral Disc Herniation in the Lumbar Spine - A Radiological Evaluation.

Objective To assess the role of facet tropism (FT) in intervertebral disc prolapse. Methods A total 98 patients with lower back pain were included in the study. Magnetic resonance imaging scans were performed and analyzed. The angles of the right and left facets were measured on the axial section. Patients without disc prolapse at the L3-L4, L4-L5 and L5-S1 levels act as controls for those with disc prolapse at the same levels. A statistical analysis was also performed. Results The incidence of FT at the L3-L4 level was of 85.2% in patients with disc herniation ( n = 27), and of 56.3% in the control group, which was statistically significant ( p = 0.008). Similarly, at the L4-L5 level, incidence of FT among cases and controls was of 71.4% ( n = 35) and 52.4% respectively ( p = 0.066). At the L5-S1 the incidence was of 66% and 51% among cases and controls respectively ( p = 0.13). Conclusion We found a positive association between FT and disc herniation at the L3-L4 level, but no association at the L4-L5 and L5-S1 levels.

Facet Tropism/Inclination and Its Association with Intervertebral Disc Herniation in the Lumbar Spine - A Radiological Evaluation / Tropismo/inclinação facetária e sua associação com hérnia de disco intervertebral na região lombar da coluna - Uma avaliação radiológica

Abstract Objective To assess the role of facet tropism (FT) in intervertebral disc prolapse. Methods A total 98 patients with lower back pain were included in the study. Magnetic resonance imaging scans were performed and analyzed. The angles of the right and left facets were measured on the axial section. Patients without disc prolapse at the L3-L4, L4-L5 and L5-S1 levels act as controls for those with disc prolapse at the same levels. A statistical analysis was also performed. Results The incidence of FT at the L3-L4 level was of 85.2% in patients with disc herniation (n= 27), and of 56.3% in the control group, which was statistically significant (p= 0.008). Similarly, at the L4-L5 level, incidence of FT among cases and controls was of 71.4% (n= 35) and 52.4% respectively (p= 0.066). At the L5-S1 the incidence was of 66% and 51% among cases and controls respectively (p= 0.13). Conclusion We found a positive association between FT and disc herniation at the L3-L4 level, but no association at the L4-L5 and L5-S1 levels.

Resumo Objetivo Avaliar o papel do tropismo facetário (TF) no prolapso discal intervertebral. Métodos Um total de 98 pacientes com dor lombar foram incluídos no estudo. Exames de ressonância magnética foram realizados e analisados, e os ângulos das facetas direita e esquerda foram medidos na seção axial. Os pacientes sem prolapso discal nos níveis L3-L4, L4-L5 e L5-S1 atuam como controles para aqueles com prolapso nos mesmos níveis. Fez-se também uma análise estatística. Resultados A incidência de TF no nível L3-L4 foi de 85,2% em pacientes com hérnia discal (n= 27), e de 56,3% no grupo controle, o que foi estatisticamente significativo (p= 0,008). Da mesma forma, a incidência de TF no nível L4-L5 entre casos e controles foi de 71,4% (n= 35) e 52,4%, respectivamente (p= 0,066). No nível L5-S1, a incidência foi de 66% e 51% nos caso e nos controles, respectivamente (p= 0,13). Conclusão Encontramos associação positiva entre TF e hérnia de disco no nível L3-L4, mas nenhuma associação nos níveis L4-L5 e L5-S1.

Role of Diffusion-Weighted Magnetic Resonance Imaging in Prediction of Pathological Complete Response to Neoadjuvant Chemotherapy in Locally Advanced Breast Cancer and Its Molecular Subtypes.

Purpose The aim of this study was to evaluate the role of apparent diffusion coefficient (ADC) and hence diffusion-weighted imaging in prediction of pathological complete response (pCR) to neoadjuvant chemotherapy (NACT) in locally advanced breast cancer (LABC) and its molecular subtypes. Methods In this tertiary hospital-based prospective study, 30 patients aged 30 to 65 years, having clinically/cytologically diagnosed LABC, were included. Magnetic resonance imaging (MRI) was done to obtain prechemotherapy ADC (ADC pre ), postchemotherapy ADC (ADC post ), change in ADC (ΔADC), and ΔADC% for each tumor and its subtype. Postsurgical pCR was used as the reference standard for determining tumor response. All four ADC parameters were compared between pCR and non-pCR groups. Results Of the 30 patients, 19 (63.3%) patients showed pCR, while 11 (36.7%) patients did not. The pCR group showed significantly lower mean ADC pre ( p < 0.001) and higher mean ADC post ( p < 0.05), ΔADC, and ΔADC% ( p = 0.000) than non-pCR group. The best cutoff values to differentiate responders from nonresponders with receiver operating characteristic curve analysis of ADC pre , ADC post , and ΔADC% were 0.98 × 10 -3 mm 2 /s (68.4% sensitivity, 63.6% specificity), 1.31×10 -3 mm 2 /s (68.4% sensitivity, 63.6% specificity), and 25% (84.2% sensitivity, 90.9% specificity), respectively. Human epidermal growth factor receptor 2 (HER2)-enriched subtype showed significant difference in mean ADC pre ( p = 0.045), while triple-negative subtype showed significant differences in mean ADC post ( p = 0.032) and mean ΔADC ( p = 0.019) between the two groups. Conclusion ADC pre , ADC post , and ΔADC can predict pCR to NACT in LABC. Among molecular subtypes, ADC pre was predictive only in HER2-enriched subtype, while ADC post and ΔADC were predictive only in triple-negative subtype.

Correlation of Clinical, Hormonal, Biochemical and Ultrasound Parameters Between Adult and Adolescent Polycystic Ovarian Syndrome: Adult and Adolescent PCOS.

Purpose: To correlate the clinical, hormonal, biochemical and ultrasound parameters in adolescent patients with polycystic ovarian syndrome (PCOS) and to compare them with adult patients. Methods: This was a prospective, correlational study. 50 adult (20-35 years) and 50 adolescent patients (15-19 years) who had features of PCOS (Rotterdam Criteria, 2003) were selected. The control group comprised of 50 women of same age in each group with normal parameters. Pelvic ultrasound was done in early follicular phase (3-5th day of menstrual cycle). Assessment of hormonal and biochemical parameters (LH/FSH ratio, free testosterone level, lipid profile and fasting glucose/insulin ratio) and grey-scale ultrasound was done. Results: No significant difference was observed in menstrual pattern in adults and adolescents with PCOS. The mean values of serum LH/FSH ratio and free testosterone were significantly higher in both adult and adolescent PCOS patients as compared to their controls (p < 0.001). The mean value of serum insulin was significantly higher (p < 0.001) with positive correlation (adult: r = 0.655, p < 0.01; adolescent: r = 0.451, p < 0.01) of serum insulin with free testosterone. Hyperandrogenemia without hyperinsulinemia was found in 56% adolescent and 60% adult PCOS patients. 82% adolescent and 88% adult PCOS patients showed multiple follicles (> 5) on ultrasound. The ovarian morphology had positive correlation with serum LH and free testosterone. The mean ovarian volume was significantly higher in adult (10.48 ± 4.38 vs. 4.17 ± 0.91) and adolescent (11.08 ± 5.82 vs. 4.23 ± 0.89) PCOS patients, when compared with controls, respectively. Conclusion: No statistically significant difference was noted in PCOS between adults and adolescents.

Diagnosis of Genital Tuberculosis in Infertile Women by Using the Composite Reference Standard.

Female genital tuberculosis (FGTB) can be asymptomatic or even masquerade as other gynecological conditions. Conventional methods of FGTB diagnosis include various imaging, bacteriological, molecular, and pathological techniques that are only positive in a small percentage of patients, leaving many cases with undiagnosed condition. In the absence of a perfect diagnostic method, composite reference standards (CRSs) have been advocated in this diagnostic study. This study assesses the agreement between traditional diagnostic modalities using CRS and prevalent TB groups among different fallopian tube infertility manifestations. A total of 86 women with primary and secondary infertility were included in the study and subjected to bacteriological, pathological, and radiological examination for the diagnosis of FGTB. Results were evaluated statistically for concordance of the diagnostic tests to the CRS by sensitivity and specificity, while PPV and NPV were calculated for the performance of diagnostic tests of FGTB. We observed that 11.2% of women were found to be true positives by means of CRS. The positive findings by CRS were as follows: ultrasonography (13.9%), laparoscopy (14%), hysteroscopy (12%), GeneXpert (4.8%), culture (4.8%), polymerase chain reaction (4.8%), and histopathology (6.4%). GeneXpert and culture were found to have a perfect agreement with CRS. Hysterosalpingography, laparoscopy, and hysteroscopy have a fair agreement with CRS. Out of 43 women with tubal factor infertility, 6 women were found in the definitive TB group with mixed conditions of tubal manifestations. This study evaluates and demonstrates the reliability of the collective assessment of various diagnostic methods with CRS findings that help in identifying different TB groups of genital tuberculosis patients from all infertile patients by applying the criteria of CRS.

An Atypical Case of Thrombotic Microangiopathy Secondary to Acute Pancreatitis.

Thrombotic microangiopathies (TMAs) are a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end-organ damage. It can often be challenging to determine the underlying etiology. Our patient presented with acute pancreatitis and later developed thrombocytopenia and hemolytic anemia, along with acute renal failure. A working diagnosis of an atypical hemolytic uremic syndrome was made; however, he improved clinically and eculizumab was not started. Workup for the atypical hemolytic uremic syndrome was unrevealing. The authors propose that the pancreatitis triggered a secondary TMA, which although rare, has previously been described in the literature. This case illustrates the diagnostic and therapeutic challenges associated with TMAs.

Risk factors for vitamin D deficiency in sickle cell disease.

Vitamin D deficiency (VDD), 25-OHD levels <20 ng/ml, is prevalent among patients with sickle cell disease (SCD) and is linked to acute and chronic pain and bone fracture in this population. There is limited literature regarding VDD-associated risk factors for SCD. We examined potential clinical and genomic parameters associated with VDD in 335 adults with SCD in a cross-sectional study. VDD was present in 65% of adult SCD patients, and 25-OHD levels independently and positively correlated with older age (P < 0·001) and vitamin D supplementation (P < 0·001). 25-OHD levels were higher in SCD patients over 40 years of age compared to the general African-American population. Both lower 25-OHD levels and increased pain frequency were associated with increased expression of SLC6A5 encoding glycine transporter-2 (GlyT2), a protein involved in neuronal pain pathways. Lower 25-OHD levels were also associated with increased expression of CYP3A4, and with decreased expression of GC (also termed DBP) and VDR, three genes involved in vitamin D metabolism. We conclude that vitamin D supplementation should be an almost universal feature of the care of young adults with SCD, and that further research is warranted into genomic factors that regulate vitamin D metabolism in SCD.