A Case of Autosplenectomy in Sickle Cell Trait Following an Exposure to High Altitude.

A 24-year-old man presented with acute abdominal pain upon ascent to moderate altitude (3500 m). An immediate evaluation revealed a splenic infarct, and he was evacuated to sea level. Upon recovery, he was sent back to 3500 m without detailed etiological evaluation, whereupon he experienced recurrent episodes of left-side subcostal pain.

Cardiac tamponade causing severe reversible hyponatraemia.

Severe hyponatraemia in setting of cardiac tamponade is very rare and only few case reports have been reported so far. This case report highlights pericardial tamponade as a rare but easily treatable cause of severe hyponatraemia. Pertinent literature is also reviewed.

Role of Arsenic Trioxide in the Management of Aplastic Anemia.

Aplastic anemia is a common problem in the developing world with therapeutic challenges as most of the patients are non-affording to standard care owing to resource constraints. We present the results of an open label single arm, non-randomized, single center, prospective phase II trial of the compassionate use of arsenic trioxide in patients refractory to ATG or unable to afford the standard of care therapy. The study was prematurely terminated at eighth week due to non-response in 100% of patients and death in two patients.

AYA-Myeloma: Real-World, Single-Center Experience Over Last 5 Years.

Introduction: Multiple myeloma (MM) is considered as a disease of the old with the reported median age of 60-70 years. The disease occurred a decade earlier in the Indian subcontinent. The literature on MM in adolescents and young adult (AYA) is limited.

Use of Surrogate Samples to Monitor pH During High dose Methotrexate Therapy.

High dose methotrexate (Mtx) therapy is commonly used in hemato-oncological practice. Alkalization of urine is a part of high dose methotrexate therapy for preventing crystallization in urine to avert renal insufficiency. Alkalization is monitored by urine pH at regular intervals.

Phenotypic Diversity of Sickle Cell Disease in Patients with a Double Heterozygosity for Hb S and Hb D-Punjab.

Phenotypic heterogeneity for sickle cell disease is associated to several genetic factors such as genotype for sickle cell disease, β-globin gene cluster haplotypes and Hb F levels. The coinheritance of Hb S (HBB: c.20A > T) and Hb D-Punjab (HBB: c.

Amputations in Sickle Cell Disease: Case Series and Literature Review.

In this study, we describe four new patients with sickle cell disease who had limb amputations. Two of the patients had sickle cell anemia [Hb S (HBB: c.20A > T) (β(S)/β(S))] with refractory leg ulcers that required amputations.

Genital vasculitis secondary to all-trans-retinoic-acid.

Acute promyelocytic leukaemia is among the most curable haematological malignancies after the introduction of differentiating agents (arsenic trioxide (ATO) and all-trans-retinoic-acid (ATRA)). Despite excellent cure rates, approaching 85-95% in various series, APL is associated with significant early mortality and morbidity. ATRA-related side effects partly contribute to this morbidity, which commonly presents as differentiation syndrome, pseudo tumour cerebri, dermatitis, gastrointestinal disorders, liver dysfunction (raised transaminases) and dryness of skin/eyes.

Risk Factors of Pulmonary Hypertension in Brazilian Patients with Sickle Cell Anemia.

This study was a prospective cross-sectional cohort study of 125 patients with sickle cell anemia (SS) between the ages of 16 to 60 years. Enrolled patients were followed-up prospectively for 15 months. Demographic, clinical, hematological and routine biochemical data were obtained on all patients.

Deep venous thrombosis in children with sickle cell disease.

Background: Deep venous thrombosis (DVT) is rare in children compared to adults. Its incidence and risk factors in children are not well known. This study determined these aspects of DVT in children with sickle cell disease (SCD).

Treatment of the acute sickle cell vaso-occlusive crisis in the Emergency Department: a Brazilian method of switching from intravenous to oral morphine.

Objectives: Describe the treatment of patients with vaso-occlusive crises (VOC) in a Brazilian emergency department (ED) and the successful switch from intravenous to oral morphine.

Patients And Methods: We analyzed records of 315 patients with sickle cell disease using two different protocols for pain: one in March 2010 prescribing intravenous morphine every 4 h throughout their stay, and another in March 2011 and 2012 prescribing one initial dose of intravenous morphine followed by equianalgesic doses of oral morphine every 4 h. Patients were triaged into three groups: mild, moderate, and severe VOC.

Matched unrelated donor allogeneic transplantation provides comparable long-term outcome to HLA-identical sibling transplantation in relapsed diffuse large B-cell lymphoma.

The objective of this retrospective analysis was to compare outcomes of patients with diffuse large B-cell lymphoma (DLBCL) who received either a matched sibling (sib) or an unrelated donor (URD) allogeneic hematopoietic cell transplantation (allo-HCT). Long-term outcome of 172 DLBCL patients receiving URD-HCT between 2000 and 2007 and reported to the European Group for Blood and Marrow Transplantation, was compared with that of 301 subjects, allografted from sib-HCT. With a median follow-up of 45 months, 3-year PFS approached 35% for both groups; overall survival (OS) was 42% for sib-HCT versus 37% for URD (NS).

Leg amputation for an extensive, severe and intractable sickle cell anemia ulcer in a Brazilian patient.

A 35-year-old African Brazilian patient had sickle cell anemia complicated with recurrent vasoocclusive (VOC) crises and refractory painful leg ulcers for 16 years. The ulcers started over both medial malleoli and expanded gradually. The ulcer on the left leg spread from the foot to the knee circumferentially and was refractory to all forms of therapy within the frame work of multi-disciplinary care.

Newborn screening program for hemoglobinopathies in Rio de Janeiro, Brazil.

Background: Newborn screening for hemoglobinopathy in Brazil has been decentralized until 2001 when the Health Ministry of Brazil established the National Newborn Hemoglobinopathy Screening Program. The State of Rio de Janeiro started a program in collaboration with the State Health Department and the Institute of Hematology in Rio (HEMORIO). The goal of this study was to evaluate the effectiveness of the first 10 years of the Newborn Hemoglobinopathy Screening Program in identifying and managing infants with Sickle cell disease (SCD) in the State of Rio de Janeiro.

Hematopoietic transplantation from adult unrelated donors as treatment for acute myeloid leukemia.

Transplantation from unrelated donors (URD) is increasingly being used as treatment for hematological malignancies, including acute myeloid leukemia (AML). This increase is the consequence of the availability of more than 11 million URD volunteers and the more efficient donor search process in the recent years. Median time to identify a suitable URD is now 2 months.

Autologous and allogeneic stem cell transplantation in Hodgkin's lymphoma.

Newly diagnosed patients who have advanced-stage Hodgkin's lymphoma have an excellent prognosis because most of them can be cured with initial treatment. In contrast, the prognosis for patients relapsing after first-line therapy with either combination chemotherapy or chemotherapy followed by radiotherapy remains poor in many cases. In most of these cases, high-dose chemotherapy and autologous stem cell transplantation (ASCT) is currently considered to be the treatment of choice.

Prognostic indexes in follicular lymphoma: a comparison of different prognostic systems.

Background: The International Prognostic Index (IPI), initially designed for aggressive lymphomas, is also used in follicular lymphoma (FL) and other indolent lymphomas. Two new prognostic indexes have recently been proposed for FL [the Italian Lymphoma Intergroup (ILI) Index and the Follicular Lymphoma International Prognostic Index (FLIPI)].

Patients And Methods: Three indexes, IPI [age >60 years, extranodal involvement two or more sites, elevated lactate dehydrogenase (LDH), Eastern Cooperative Oncology Group performance status > or =2, stage > or =3], ILI (age >60 years, extranodal involvement two or more sites, elevated LDH, male sex, B symptoms, erythrocyte sedimentation rate > or =30 mm first hour) and FLIPI (age >60 years, stage > or =3, elevated LDH, nodal involvement five or more, haemoglobin level < or =12 g/dl) were calculated in 411 patients with FL.