Association of anti-HCV sero-prevalence with blood transfusion and practice of haemodialysis from multiple centres in patients on maintenance haemodialysis.

OBJECTIVES: To determine the frequency of anti-HCV in patients on maintenance haemodialysis (HD) and its association with history of blood transfusion and with the practice of HD from more than one center. METHODS: All the patients on maintenance HD at Bahria International hospital (BIH) Rawalpindi from March 2019 to May 2019 were included. Demographic details, history of blood transfusions and history of HD from any other center in addition to BIH, were recorded. Anti-HCV was done by chemiluminescent assay. Chi-square was used to compare the categorical variables. Odds ratio (OR) and relative risk (RR) for the groups exposed to risk were calculated. RESULTS: Of 96 patients, 40 (41.6%) were anti-HCV positive. Sixty-two (64.6%) had transfusion history. Thirty-one (50%) of these 62 were anti-HCV positive as compared to 9 (26.5%) of 34 with no history of transfusion (p=0.025); OR=2.78 (p=0.0278), RR=1.89 (p=0.0420). Among 66(68.7%) of 96 who had HD from other centres in addition to ours, 33(50%) were anti-HCV positive as compared to 7(23.3%) of 23 who had HD from BIH only (p=0.014); OR=3.29 (p=0.0167), RR=2.14 (p=0.0309). CONCLUSION: There was a high prevalence (41.6%) of anti-HCV in our HD patients and anti-HCV positivity had significant association with history of blood transfusion as well as with history of HD from multiple centres.

Postmortem Findings in Fatal Dengue Haemorrhagic Fever.

During recent epidemic of dengue fever in Peshawar, there has been a significant increase in dengue-associated admission in hospitals. We performed a postmortem examination of dengue-related death in our hospital. He was a young man, who was given supportive care at our tertiary care hospital, despite that he died on seventh day of admission. The autopsy findings in this case included multiple organ involvement including heart, lungs, kidneys and brain. There was histopathological finding of microabscesses in heart, lungs, brain and kidneys, which have not been reported in the literature yet. In patients with severe dengue, deaths do occur despite current supportive management. Knowing the pathological processes involved in severe disease, it can help identify early predictors of disease severity and help applying adequate clinical interventions.

Delta-Beta Thalassaemia in a Pathan Family.

Delta-beta-thalassaemia (δß-thalassaemia) is a rare type of thalassaemia which mostly results from deletion of δ and ß genes with preservation of γ genes. δß-thalassaemia is classified into (δß)+ and (δß)0 types. The (δß)0-thalassemia is further divided into GγAγ(δß)0-thalassaemia and Gγ(Aγδß)0-thalassaemia. In heterozygous state, (δß)0mutations give rise to phenotype resembling ß-thalassaemia trait but with raised Hb-F, ranging from 5 to 20%, without a rise in Hb-A2. In homozygotes, the clinical picture is usually that of thalassaemia intermedia and the patients have 100% Hb-F. Workup of a 1-year child suffering from pallor, chronic ill health, and splenomegaly referred to our laboratory with the suspicion of ß-thalassaemia, ultimately resulted in a diagnosis on polymerase chain reaction as having homozygous inversion/deletion Gγ(Aγδß)0-thalassaemia. Her family members were also investigated.

Clinico-Haematological Features of Paroxysmal Nocturnal Haemoglobinuria.

The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria (PNH). It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 (81.8%) males and 4 (18.1%) females. Median age was 27 years. Pallor, fever, fatigability and haemoglobinuria were the most common clinical features. Pancytopenia was seen in 13 (59.09%) and hypocellular marrow was found in 14 (63.6%) patients. One patient presented with Budd Chiari syndrome.

DRY TAP: A DIAGNOSTIC ALERT FOR UNDERLYING BONE MARROW PATHOLOGY.

BACKGROUND: Dry tap is an annoying experience in bone marrow (BM) findings, especially in cases where the diagnosis may hinge on BM findings. This study was conducted to determine, on, the basis of bone marrow (BM) trephine biopsy, the frequency of various underlying conditions causing a dry tap, among different age groups. METHODS: It was a descriptive study carried out at PAF hospital Mianwali, Pakistan from 1" Jan 2009 to 31 Dec 2012. Record of all BM aspirations and trephine biopsies performed during 4 years was retrieved from hospital's laboratory. Total number of BM aspirations and trephines were counted and the subject's ages and genders recorded. Frequencies and percentages of patients with dry tap, in paediatric group (<15 years of age), young to middle-aged group (15-59 years) and the elderly (> or = 60 years) were calculated. Diagnoses of patients with dry tap made on BM biopsy were noted for each group and their frequencies calculated. RESULTS: Of 548 BM aspirations, dry tap was encountered in 52 (9.5%) cases. Acute lymphoblastic leukaemia (ALL) was the commonest cause of dry tap in paediatric age, seen in 6 (60%) of 10 children. In young to middle-aged group, non Hodgkin lymphoma (NHL) was the commonest cause, found in 6 (30%) of 20 cases. NHL and metastatic tumours, seen in 8 (36.4%) and 6 (27.3%) of 22 patients respectively, were the most frequent causes of dry tap in the elderly. CONCLUSION: Dry tap, in most of the cases, is like a diagnostic alert for the presence of an underlying BM pathology, nature of which depends upon age group.

Retinoblastoma in an adult.

BACKGROUND: Retinoblastoma is the most common pediatric ocular tumour. It may rarely present in adults. The present case adds to the number of 26 cases already published in literature since 1919 till 2013. Our aim is to highlight the rare occurrence of retinoblastoma in adults along with its features which differentiate it from paediatric retinoblastoma. CASE PRESENTATION: We describe a case of adult onset retinoblastoma (group E, according to the international classification of retinoblastoma) occurring in a 25 year old male. He presented with decreasing visual acuity in the right eye of 4 months duration. He had neo-vascular glaucoma and pseudohypopyon. B scan ultrsonography of his right eye showed intraocular growth without any calcification. The CT scan of the orbits and brain showed intraocular growth in the right eye with no calcification. Enucleation of the right eye was carried out. Retinoblastoma was confirmed on histopathology of the enuleated globe. CONCLUSIONS: The present case adds to the number of adult Rb patients reported in literature. Early detection to salvage the life can be made possible if the clinician keeps a high index of suspicion when observing retinal mass of adult onset. Proper counselling of the patient in order to seek his full involvement in management may help in improving the prognosis of the disease.

Severe thalassaemia intermedia with multiple fractures: role of transfusion therapy.

Thalassaemia intermedia includes thalassaemias with clinical severity intermediate between asymptomatic thalassaemia minor and transfusion dependent thalassaemia major. By definition patients of thalassaemia intermedia maintain a haemoglobin level of 7-10 g/dl and do not, or only occasionally, require blood transfusion. An eight-year-old girl who was a known case of thalassaemia intermedia and had been occasionally transfused presented with fever, pain and swelling over the wrists, ankles and above the right knee joint. Radiographs showed medullary widening, cortical thinning and; multiple, recent and old, partially healed fractures of metadiaphseal regions of long bones. Her fractures have been immobilized by means of back slabs. In view of her recurrent fractures and growth retardation we advised a regular transfusion-chelation regimen to our patient to suppress her ineffective dyserythropoiesis. The treatment is expected to prevent further bone fragility and fractures, as well as improve her life quality.

A case of systemic lupus erythematosus with aplastic anaemia.

Systemic lupus erythematosus is an autoimmune disorder, which has a rare association with aplastic anaemia. A young 26 years old lady who presented with a history of intermittent fever, microcytic anaemia, joint pains and mild degree of splenomegaly was investigated. Bone marrow examination showed aplasia. Serological tests revealed positive antinuclear antibody and anti double-stranded DNA tests. Patient was diagnosed as having aplastic anaemia with Systemic lupus erythematosus, managed with steroids and being followed up for monitoring the response.