Autoimmune Hemolytic Anemia: A Challenging Complication of Allogeneic Hematopoietic Stem Cell Transplant.

Autoimmune hemolytic anemia (AIHA) is an uncommon but recognized complication of a hematopoietic stem cell transplant (HSCT). Management challenges include the absence of established guidelines and variable response rates to first-line treatments. We present a case series of three patients, all submitted to HSCT for non-oncologic diseases, who developed AIHA as a complication in the following months.

Neonatal Thrombocytopenia Due to Dual Alloimmune and Autoimmune Mechanisms.

Alloantibody-mediated and autoantibody-mediated immune destruction are common causes of early neonatal thrombocytopenia. The authors report a case of severe, early-onset thrombocytopenia with mucocutaneous bleeding in an otherwise well-appearing full-term neonate. Recurrence of thrombocytopenia following initial treatment and its persistence after 2 weeks of life suggested a dual immune mechanism.

Nephrotic syndrome on sickle cell disease: the impact of Hydroxyurea.

Nephropathy is a common under-recognised complication of sickle cell disease (SCD) and one of the main factors of poor prognosis in these patients. The association between nephrotic syndrome and SCD in children is rare. Strategies for sickle cell nephropathy prevention are still poorly established.

Use of gabapentin in the treatment of chronic pain in an adolescent with sickle cell disease.

Vaso-occlusive crises are the most common manifestation of sickle cell disease (SCD) and the main cause of hospital admission in these patients. There is emerging evidence that vaso-occlusive pain has both nociceptive and neuropathic components. However, the treatment of SCD-related pain with neuropathic drugs has not yet been systematically studied, particularly in children.

An unusual cause of neonatal cyanosis….

We report a case of a female neonate whose pulse oximetry screening for congenital heart disease at 40 h of life was positive. The pregnancy was uneventful with no relevant family history. The neonate presented with bluish discolouration of the skin lasting until day 15.

Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes.

Background: Sickle cell disease (SCD) has extremely variable phenotypes, and several factors have been associated with the severity of the disease.

Objectives: To analyze the chronic complications of SCD and look for predictive risk factors for increased severity and number of complications.

Methods: Retrospective study including all children followed for SCD in the Paediatric Haematology Unit of a tertiary hospital in Portugal, who completed 17 yr old between the years 2004 and 2013.

Hepatitis-associated aplastic anaemia: a poor prognosis.

A 13-year-old boy presented with spontaneous skin and mucosal bleeds 3 weeks after acute hepatitis of unknown aetiology. Laboratory analyses revealed pancytopenia and bone marrow biopsy that confirmed the diagnosis of aplastic anaemia. Other causes of congenital and acquired aplastic anaemia were excluded.

Severe congenital thrombocytopaenia--first clinical manifestation of Noonan syndrome.

This report focuses on a male infant, the first born of non-consanguineous parents diagnosed with polyhydramnios at 26 weeks of gestation. The newborn was admitted during the neonatal period with bleeding diathesis associated with a low platelet count at birth (5×10(9)/l).The authors registered a persistent low platelet count (9000-129 000/l) during the infants 1st year of life.