Introduction: Multiply transfused patients are frequently subjected to platelet alloimmunization. These platelet alloantibodies produced can result in refractoriness to platelet transfusion. Material and Methods: Ninety five thrombocytopenic (platelet count
OBJECTIVE: Nocardia kroppenstedtii was isolated from the spinal vertebral abscess of a 78-year-old patient presenting with mid-thoracic pain and bilateral lower limb weakness and numbness. The patient was on long-term immunosuppressive therapy with steroids for underlying autoimmune hemolytic anemia. Investigations showed a T5 pathological fracture and vertebra plana with the erosion of the superior and inferior endplates. There was evidence of paraspinal collection from the T4-T6 vertebrae with an extension into the spinal canal. Analysis of Nocardia 16S rRNA (99.9%, 1395/1396 nt) and secA1 gene (99.5%, 429/431 nt) fragments showed the highest sequence similarity with Nocardia kroppenstedtii type strain (DQ157924), and next with Nocardia farcinica (Z36936). The patient was treated with intravenous carbapenem and oral trimethoprim-sulfamethoxazole for four weeks, followed by another six months of oral trimethoprim-sulfamethoxazole. Despite the improvement of neurological deficits, the patient required assistive devices to ambulate at discharge. This study reports the first isolation of N. kroppenstedtii from the spinal vertebral abscess of a patient from Asia. Infections caused by N. kroppenstedtii may be underdiagnosed as the bacterium can be misidentified as N. farcinica in the absence of molecular tests in the clinical laboratory.
Idiopathic hypereosinophilic syndrome (HES) is an uncommon disorder which usually presents with prolonged and significant primary eosinophilia with end-organ dysfunction. Damaging proteins released by the eosinophilic granules are responsible for the tissues and organ system damage. Here we report two cases of idiopathic HES. Both the patients were young lady presented with high grade fever and concomitant symptoms. Laboratory findings showed leucocytosis with predominant neutrophilia and marked eosinophilia. A diagnosis of idiopathic HES was made after excluding secondary causes of eosinophilia. However, the first patient was complicated with multiple venous thrombosis and intravenous heparin was started which was later changed to subcutaneous low molecular weight heparin (LMWH). The patient developed pleural effusion and consolidation. Intravenous Tazoscin, tablet Prednisolone and tablet Hydroxyurea was started and the patient responded well. Despite treatment, two weeks later, suddenly the patient collapsed and unfortunately succumbed. On the other hand, the second patient was complicated with fever, thrombocytopenia, haemolytic anaemia, acute renal failure and neurological deficit which were part and parcel of thrombotic thrombocytopenic purpura (TTP). Plasma exchange was commenced and patient’s condition had slowly improved. Nevertheless, the hypoxia which she sustained during the multiple episodes of fits had resulted in permanent brain injury and thus requiring a tracheostomy for prolonged ventilatory support. Currently, there is no cure for HES. The main aim of treatment is to minimise the tissue damage caused by the hypereosinophilia. Early diagnosis and intervention are therefore crucial in preventing the spread of the disease and the end-organ damage.
INTRODUCTION: Glycohemoglobin (HbA1c) most accurately reflects the previous two to three months of glycaemic control. HbA1c should be measured regularly in all patients with diabetes, and values should be maintained below 7% to prevent the risk of chronic complications. Apart from the genetic variants of haemoglobins many other conditions also known to affect HbA1c measurements. In this study we evaluated the conditions that cause low HbA1c results.
METHODS AND MATERIALS: The data was collected retrospectively HbA1c was measured in our laboratory by Biorad Variant II turbo 2.0. The method is based on chromatographic separation of HbA1c on a cation exchange cartridge. This method has been certified by National Glycohemoglobin Standardization Programme (NGSP). 58437 requests were received in a period of one year (January to December 2011). Medical records were reviewed to identify the conditions that might be associated with these low values.
RESULTS: Among 58437 samples analysed, 53 patients had HbA1c levels < 4.0%. Fourteen patients had haemoglobinopathy. In 34 patients without Hb variants had conditions such as chronic liver disease, chronic kidney disease, haemolytic anaemia, pregnancy, and anaemia of chronic disease. Five non-pregnant individuals who were screened for diabetes mellitus had HbA1c levels < 4%.
CONCLUSION: Our study underscores the importance of that both laboratories and the physicians should be aware of the factors that can influence the HbA1c results. The haematological status should be taken into consideration for proper interpretation of HbA1c results.
Familial distal renal tubular acidosis (dRTA) can be caused by mutations in the Cl2/HCO32 exchanger of the renal Type A intercalated cell, kidney AE1/SLC4A1. dRTA-associated AE1 mutations have been reported in families from North America, Europe, Thailand, Malaysia, Papua-New Guinea, Taiwan, and the Philippines, but not India. The dRTA mutation AE1 A858D has been detected only in the context of compound heterozygosity. We report here two unrelated Indian patients with combined hemolytic anemia and dRTA who share homozygous A858D mutations of the AE1/SLC4A1 gene. The mutation creates a novel restriction site that is validated for diagnostic screening.
Mycoplasma pneumonia is a common cause of respiratory disease and more so in school going children. The spectrum of the manifestations range from haematological, dermatological, neurological, musculoskeletal, renal, cardiac and also gastrointestinal. The treatment approach has varied over time. In this report we would like to share our experience in a case of M.pneumonia with autoimmune haemolytic anaemia (AIHA).
In a survey of over 1,000 patients with leprosy, 47 cases ( 4.4 per cent) were found to have glucose-6-phosphate dehydrogenase deficiency. A controlled clinical study suggests that such a deficiency does not modify the overall response to therapy but may predispose to a greater tendency to leprosy reactions. All patients were receiving 600 to 800 mgm. of sulphone per week and none had a frank haemolytic anaemia.
A rare syndrome of hypertension, seizures and intracranial bleed has been reported among patients with congenital hemolytic anemia who underwent multiple blood transfusions. We report this syndrome in a 12-year-old Malay girl with hemoglobin E-beta-thalassemia, who underwent intensive transfusion and subsequently had headache, visual loss, severe hypertension and seizures. A comprehensive literature review revealed 30 patients with this syndrome, of whom 15 had intracranial bleed and 12 among these 15 died. A less-intensive transfusion regimen among patients with chronic hemolytic anemia and prompt detection and management of hypertension may prevent this potentially fatal syndrome.
From 1981 to 1989, 12 patients of the University Hospital, Kuala Lumpur, were diagnosed to have Evans syndrome based on direct antiglobulin test (DAT) positive haemolytic anaemia and immune thrombocytopenia occurring either simultaneously (7 patients) or consecutively (5 patients). Their mean age at presentation was 24.8 years with a marked female preponderance. All 12 patients were given high dose steroid after diagnosis. Subsequently, other modalities including intravenous immunoglobulin (1 patient) and high dose methylprednisolone (1 patient) were given. Three patients died of intracranial haemorrhage during the first admission while 1 patient died of pulmonary embolism six months after diagnosis. Three patients had splenectomy because of thrombocytopenia. Six patients tested positive for antinuclear factor and antibodies to double stranded DNA and four of them died. Positive serology appeared to be associated with a poorer prognosis. Follow up observations indicate that patients who survive the acute attacks fare reasonably well.
A young primigravida had idiopathic warm antibody (IgG) autoimmune haemolytic anaemia (AIHA) occurring in the third trimester of pregnancy. Her haemolytic process was responsive to steroid therapy and no transfusion was needed. She delivered a healthy baby with no evidence to haemolysis, though his red cells were coated with IgG which was probably of maternal origin.
Brucellosis is a rare zoonotic infection caused by small, fastidious Gram-negative coccobacilli of the genus Brucella that may be associated with haemolytic complications including thrombotic microangiopathy and haemolytic anaemia. We describe a patient with culture confirmed brucellosis who presented with malaise, high grade fever, hepatosplenomegaly and Coombs-positive autoimmune haemolytic anaemia. The patient was successfully treated with combination of doxycycline and rifampicin with no further episodes of relapses or haemolysis. Although rare, the possibility of brucellosis should always be kept in mind in patients with risk factors who present with haemolysis and endemic area.
A study of 23 patients with Hb H disease and their 82 relatives in 17 families showed that 2 types of this condition exist. One is associated with the presence of a small slow-moving component, which we tentatively called the X component and which was invariably present in one parent. Some siblings also had it. The other type was not associated with this component. Two patients without X component had a newborn with Bart’s haemoglobin without X component. None of the parents of 20 newborns with Hb Bart’s without the X component had the X component. It was present in only one parent of each of 2 newborns with Hb Bart’s and the X component. They are thought to represent Hb H disease in the newborn period. We suggest that at least 3 abnormal genes may lead to Hb H disease, which results when 2 of the 3 combine. Severity of clinical and haematological symptoms depends upon which abnormal gene is present and which 2 are involved in any particular combination.
Key Words: a-Thalassaemia; Haemoglobin Bart’s; Haemoglobin H disease; Haemoglobinopathies
A 56-year-old Chinese lady with valvular heart disease and atrial fibrillation was referred to us from a private hospital for further management of autoimmune haemolytic anaemia. Physical examination and laboratory investigations did not support the diagnosis of haemolytic anaemia. However, direct antiglobulin test (DAT) was strongly positive with anti-IgG and negative with anti-C3d. There was also mild anaemia and reticulocytosis, which was attributable to persistent haematuria. The DAT became positive after commencing Unasyn and cessation was associated with decreasing reactivity of the positive DAT. We believe that the positive DAT in this patient was most likely due to the Unasyn therapy.