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).
Acne is one of the most common disorders affecting mankind. Although acne does not cause death, it however produces a lot of discomfort, disfigurement and psychological trauma, particularly in teenagers. Acne vulgaris is a chronic condition involving the pilosebaceous unit of the skin. It is characterised by the presence of comedones, inflammatory papules, pustules or cysts, and eventually by scarring. The end result of acne varies from hyperpigmentation, slight pitting, to extremely disfiguring scars that may develop into keloids. Acne fulminans is a rare disorder and is characterised by sudden explosive appearance of highly inflammatory, tender, crusted, ulcerated lesions involving the back, chest and face. It is one of the most scarring acute dermatologic disorders of young people. A case of acne fulminans in a young female who developed haemolysis due to dapsone is reported here.
BACKGROUND: Hypercalcemia is common in primary hyperparathyroidism malignancies and even in tuberculosis. Interestingly, systemic lupus erythematosus (SLE) rarely presents with hypercalcemia.
CASE REPORT: We describe an interesting case of SLE in a patient who was otherwise thought to have either tuberculosis or a malignancy. The patient initially presented with feeling unwell, with generalized lymphadenopathy, bilateral pleural effusion, and bilateral corneal calcium deposits secondary to severe hypercalcemia. The diagnosis of SLE was made based on positivity of antinuclear antibodies (ANA) and anti-dsDNA, the presence of serositis, lymphadenopathy, autoimmune hemolytic anemia, and constitutional symptoms. She was treated with steroids, with tremendous improvement in her general well-being, resolution of lymphadenopathy and pleural effusion, and normalization of her hemoglobin and serum calcium. The atypical presentation of SLE with hypercalcemia with pleural effusion is discussed.
CONCLUSIONS: SLE should be one of the differential diagnoses in patients presenting with severe hypercalcemia.
KEYWORDS: atypical presentation; hypercalcemia; systemic lupus erythematosus
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.
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.
Cerebral Venous Thrombosis in patients with Evan’s Syndrome of autoimmune hemolytic anemia is rare. The
common neurological symptoms are headaches, vision loss, dyslexia without agraphia, motor aphasia,
unilateral upper limb weakness and papilloedema. We present a case report of a lady with a known case of
Evan’s Syndrome whom presented with severe anemia and unilateral right sided hemiparesis with right facial
weakness. Plain and Contrast enhanced CT brain showed bilateral high parietal white matter edema with
venous thrombosis in the right transverse and superior sagittal venous sinuses. At the time of the diagnosis,
she was in hematological remission.
The presence of serum cold agglutinin can be the initial presentation of lymphoproliferative diseases. Conditions with persistent cold agglutinins are a spectrum of diseases that vary from benign lymphoproliferation of the "autoimmune-like chronic cold agglutinin disease" to malignant lymphoma. We report a case of a 72-year-old woman who presented with severe anaemia, hepatosplenomegaly and episodes of peripheral haemagglutination precipitated by cold exposure. The haemoglobin was 5.6 g/dL with a cold agglutinin titer of 1:256 at 4 degrees C and 1:8 at room temperature (30 degrees C). The cold agglutinin showed anti-I specificity and kappa light chain restriction. Peripheral blood showed atypical lymphoid cells with a B-cell immunophenotype. Immunoglobulin gene rearrangement study by polymerase chain reaction (PCR) showed an amplified band at 100 bp, consistent with a clonal proliferation of B-lymphocytes. We believe that our patient had cold antibody haemolytic anaemia as the initial presentation of a low-grade non-Hodgkin's lymphoma. The association of cold antibody haemolytic anaemia with low-grade B-cell lymphoma is unusual.
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.
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 2-year-old Chinese boy was referred to Hospital UKM for investigation of recurrent episodes of dark-coloured urine and pallor since birth. He was born prematurely at 34 weeks gestation and developed severe early-onset neonatal jaundice requiring exchange blood transfusion. Screening at birth showed Glucose-6-phosphate dehydrogenase (G6PD) deficiency. On admission, physical examination revealed pallor, jaundice and mild hepatomegaly. Results of laboratory investigations showed a hemoglobin level of 11.0 g/dl with a hemolytic blood picture, reticulocytosis of 20% and red cell G6PD activity reported as undetectable. The patient's DNA was analysed for G6PD mutations by PCR-based techniques and DNA sequencing and results showed a 24 bp deletion of nucleotide 953-976 in the exon 9 of the G6PD gene. DNA analysis was also performed on blood samples of the patient's mother and female sibling confirming their heterozygous status, although both showed normal red cell G6PD activity levels. The patient was discharged well and his parents were appropriately advised on the condition and the importance of taking folic acid regularly. This is a first case report in Malaysia of G6PD deficiency causing chronic-hemolytic anemia. The rare 24 bp deletion causes the G6PD Nara variant, previously reported only in two other unrelated males, a Japanese and a Portuguese both with chronic hemolytic anemia.
We reported a case of a woman with no past medical illness who presented with a few days' history of fever, myalgia, arthralgia, hypochromic microcytic anaemia and thrombocytopaenia and who was nonstructural protein 1 antigen (NS1Ag)-positive. Haemolytic anaemia including full blood picture work-up revealed high reticulocyte count and haemolysis with positive direct Coombs test. She was started on prednisolone and was discharged well.
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.