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Mar 2014 DOI 10.14302/issn.2372-6601.jhor-13-352
O’ Sullivan JMCorresponding author
Department of Haematology, Cork University Hospital, Cork, Ireland
Variation in the lower and upper limits of haemoglobin (Hb) ranges will alter diagnosis and referral rates of anaemia or erythrocytosis. The haemoglobin ranges for adults in Irish laboratories were applied in the context of haemoglobin levels of a representative sample of Irish adults (SLAN). Between 2.8% and 8.5% of men would be diagnosed with anaemia in different laboratories for lower limit between 130-140g/L. For women, diagnosis of anaemia would range from 0.7% to 7.3% for the lower limit between 110-125g/L. Similar reference range variations occur in UK laboratories. A suggested “normal reference range” is extrapolated from the SLAN cohort.
Mar 2026 DOI 10.14302/issn.2372-6601.jhor-25-5938
Regan CaineCorresponding author
Acquired haemophilia (AHA) is a rare coagulation disorder secondary to autoantibodies against coagulation factor, most commonly factor VIII with potential for life threatening bleeding episodes. We report a case of an 88-year-old female presenting with frank haematuria three weeks after catheter insertion. Her background was of Alzheimer’s Dementia, Asthma and Bullous Pemphigoid for which she was on low dose maintenance prednisolone (5mg). Laboratory tests showed haemoglobin 98g/dl and partial thromboplastin time (PTT) of 60s, with corrected prothrombin time 52s. Fibrinogen 5.39. As such coagulation factors were tested which revealed factor VIII of 0%. Her case was complicated by urinary tract sepsis, as such she was treated with oral prednisolone 60mg without immunosuppressive agent usage. A pan-CT scan revealed likely mesothelioma for which she declined further investigation. This case report will describe a rare presentation of AHA associated with bullous pemphigoid and mesothelioma, complicated by infection and frailty.
Apr 2023 DOI 10.14302/issn.2372-6601.jhor-23-4530
Padaro EssohanaCorresponding author
Objective Drawing up a balance sheet of 16 years follow-up of the sole case of Diamond-Blackfan anemia diagnosed in Togo with arguments of molecular biology. Observation T.S a boy, born on 5th september 2006 has been followed up since he was three months, for Diamond-Blackfan anemia (DBA) in whom there has been found the mutation of ribosomial protein RPS19 in july 2010. It was the first observation in Subsaharian Africa. The treatment by transfusions from december 2006 to december 2022 has been associated with iron chelation through deferoxamin and promptly with corticotherapy at the dosage of 2mg/kg/day. The corticotherapy has been reduced as a consequence of corticoresistance from the fourth week, then definitely interrupted after four months. The evolution is marked by a clinical improvement with a staturo-balanced curve, and during the last control of 28th december 2022, the child was 53 kg heavy and 160 cm tall. The monthly physical tests did not reveal any signs of eventual overloading and the echocardiography of 26th december 2022 was normal. On the biological plan, the rate of the haemoglobin had been stable around 50g/l as a resultant of a transfusion each 4 to 6 weeks of red blood cell pellet. The chelation of iron had been done through deferoxamin with a monthly control of serum ferritin. That serum ferritin was 738,39ng/mg at diagnosis before the beginning of transfusions and during the follow-up, we noticed an average of 2977,3ng/ml (range 1817,1ng/ml and 4448,5ng/ml) Conclusion Thanks to the regular transfusions derived from the survey of the parameters of iron and the use of deferoxamin, we have succeded in keeping alive during sixteen years a patient who caught a disease whose evolution is unpredictable.
Jun 2018 DOI 10.14302/issn.2372-6601.jhor-18-2139
S. Afriyie-Mensah JaneCorresponding author
Department of Medicine and therapeutics, School of Medicine and Dentistry, College of Health Sciences university of Ghana, Accra, Ghana.
Pulmonary hypertension (PH) has become an increasingly recognized complication in sickle cell anaemia (SCA) and is a major cause of morbidity and mortality. Though the burden of SCA in sub-Saharan Africa is high, there is paucity of data on SCA-associated PH with little or no attention given to it in routine patient care. The current study therefore sought to determine the prevalence of PH and its associated risk factors among adult patients with SCA. This was a cross-sectional study involving 76 clinically stable, hydroxyurea-naive participants. We obtained socio-demographic and clinical history. Measurement of Tricuspid Regurgitant jet Velocity (TRV) was obtained via transthoracic echocardiography and lung function was assessed using spirometry and pulse oximetry. Other investigations were complete blood counts, free plasma haemoglobin, serum urea and creatinine. Twenty-five (32.9%) of study participants had elevated TRV (≥ 2.5m/s) on Doppler echocardiography, which was suggestive of raised pulmonary artery systolic pressure. There were significant associations between elevated TRV and steady-state haemoglobin (p < 0.001), blood urea level (p = 0.030), presence of chronic leg ulcers (p = 0.043) and oxygen saturation (p < 0.001) and these may be identifiable and modifiable risk factors for selective screening with echocardiography in a resource poor setting.