Refractory Autoimmune Hematological Presentations of Undiagnosed Tuberculosis.

Four patients who presented with autoimmune cytopenias as the sole manifestation of undiagnosed tuberculosis are described here. These were refractory to conventional immunosuppressive therapy and responded dramatically to treatment of the infection. The potential association between tuberculosis and immune hematological conditions is highlighted.

Halving Time of BCR-ABL1 in Chronic Myeloid Leukemia: Is It Better Than Day-90 Value-A Multicenter Study From South India.

Background: The 90-day BCR-ABL1 (breakpoint cluster region-Abelson 1) level has been one of the accepted milestones for predicting the molecular response in patients with chronic myeloid leukemia (CML). The rate of decline in BCR-ABL1 has been considered a better predictor of the response but has not been uniformly accepted. A paucity of evidence is available to predict the accuracy of the rate of decline in the Indian context.

Does Interim PET Scan After 2 Cycles of ABVD Predict Outcome in Hodgkin Lymphoma? Real-World Evidence.

Purpose: Escalated BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, and prednisone) improves overall survival (OS) in patients with Hodgkin lymphoma (HL) relative to ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) therapy. However, the associated higher cost and toxicity discourage clinicians from prescribing it. Identifying high-risk patients and administering escalated BEACOPP remains an effective strategy.

Transesophageal bronchoscopic ultrasound-guided fine-needle aspiration (EUS-B-FNA)-Pushing the boundaries in the diagnosis.

Isolated mediastinal adenopathy is a diagnostic challenge in the paediatric population, often requiring invasive surgical procedures for diagnosis. We describe a novel minimally invasive modality in a 20 month toddler-transesophageal bronchoscopic ultrasound-guided fine-needle aspiration (EUS-B-FNA). This is the youngest reported use of this modality, highlighting feasibility, technical issues, safety, and rapid diagnosis leading to expedited treatment.

Successful penile reconstruction following prior arteriovenous loop thrombosis due to undiagnosed protein-S deficiency and exogenous testosterone.

Flap failure from microvascular thrombotic occlusion is a rare but significant cause for unsuccessful reconstructive surgery. We encountered thrombosis of arteriovenous loop in a patient undergoing phallus reconstruction. Further investigations revealed underlying previously asymptomatic hypercoagulable state due to protein-S deficiency in addition to long-term exogenous testosterone administration.

Screening assays for primary haemophagocytic lymphohistiocytosis in children presenting with suspected macrophage activation syndrome.

Background: Primary haemophagocytic lymphohistiocytosis (HLH) screening assays are increasingly being performed in patients presenting with macrophage activation syndrome (MAS). The objective of this study was to describe their diagnostic and prognostic relevance in children who had presented to paediatric rheumatology and had undergone investigative work up for MAS.

Methods: Data was obtained retrospectively from an existing protein screening assay database and patient records.

Distribution of dyssynchrony in subjects with no known cardiac disease and comparison of velocity vector imaging to color-coded tissue Doppler imaging.

Data on the distribution of dyssynchrony in subjects with normal ejection fraction (EF) and normal QRS are scarce. We studied 100 subjects with no known cardiac disease (52% male, mean age 60 ± 17 years) using velocity vector imaging (VVI). Seventeen percent had septal to lateral (S-L) wall longitudinal delay >75 msec, 63% of subjects had S-L wall radial delay >75 msec, and 25% had a circumferential opposing wall delay >100 msec.

Prominent megakaryocytic dysplasia after regression of transient abnormal myelopoiesis associated with GATA-1 mutation.

About a quarter of children with Down syndrome and transient abnormal myelopoiesis (TAM) progress to acute megakaryoblastic leukemia (AMKL). We describe isolated dysmegakaryopoiesis despite complete resolution of TAM in an 18-month-old girl, who developed AMKL 6 months later.

Hepatic subcapsular hematoma: two neonates with disparate presentations.

Subcapsular hematoma of the liver rarely occurs in neonates and the diagnosis is often missed or delayed. We report two babies who had this uncommon condition in the early neonatal period. In the first baby, the hematoma was associated with ventouse delivery and presented with abdominal distension and worsening jaundice.

Outcome of childhood relapsed or refractory mature B-cell non-Hodgkin lymphoma and acute lymphoblastic leukemia.

Patients with childhood relapsed and refractory mature B-cell non-Hodgkin lymphoma (B-NHL) and acute lymphoblastic leukemia (B-ALL) are rare and have a dismal prognosis. The previous UK national analysis of 26 children over a 7-year period prior to 1996 had highlighted the poor outcome, with only three survivors. This 10-year multicenter study evaluated recent data, since 2000.

Clinical profile and outcome of urotheliotropic viral haemorrhagic cystitis following haematopoietic stem cell transplantation: a 7-year tertiary centre analysis.

Viral haemorrhagic cystitis (HC) is a significant complication after haematopoietic stem cell transplantation (HSCT), with a potential for major morbidity. The aim of this 7-year analysis of 1160 HSCT patients was to evaluate risk factors for the incidence, severity, toxicity of therapy, clinical course, and outcome of this condition. The overall incidence of HC was 5·8%, with most cases occurring after allogeneic HSCT.

Rheumatologic manifestations of benign and malignant haematological disorders.

Diseases of blood and lymphoreticular system can have multisystem manifestations. Rheumatologic involvement has been reported in association with many benign and malignant haematological disorders; these patients are equally likely to present to both clinical rheumatologists and haematologists. This review focuses on the well-described rheumatologic features, other occasionally reported rheumatologic manifestations and unusual musculoskeletal complications related to the treatment in patients with underlying haematological conditions.

First report of fatal human infections with the cactophilic yeast Sporopachydermia cereana.

Sporopachydermia cereana is a cactophilic yeast, which is not recognised as a human pathogen. We describe two fatal infections with this fungus in profoundly neutropenic patients. S.

Dyssynchrony in obese subjects without a history of cardiac disease using velocity vector imaging.

Background: The aim of this study was to examine the occurrence of intra-left ventricular (LV) dyssynchrony in obese versus nonobese subjects without known cardiac disease using Velocity Vector Imaging (VVI).

Methods: One hundred ninety consecutive subjects with no known cardiac disease had their echocardiograms analyzed using VVI after excluding subjects with QRS durations>120 msec or LV ejection fractions<55%. Study subjects were divided into two groups on the basis of body mass index: obese (>30 kg/m2) and nonobese (<30 kg/m2).

The feasibility of plerixafor as a second-line stem cell mobilizing agent in children.

In patients heavily pretreated with myelosuppressive chemotherapy or irradiation, Granulocyte colony stimulating factor (G-CSF) may fail to mobilize stem cells from the bone marrow. Plerixafor is emerging as a reliable alternate option in such situations in adult patients. Robust data in support of the high efficacy and safety of plerixafor are available in adults.

Epstein-Barr virus infections after allogeneic stem cell transplantation: a comparison between non-malignant and malignant hematological disorders.

Hematological cancers and non-malignant hematological disorders are biologically diverse conditions and are treated differently. We compared the pattern of EBV infections following allogeneic stem cell transplantation between the above two groups of hematological disorders. Eighty-three transplants were evaluated over a consecutive 7-year period at a single center.