MyMedR

Notes of a case of hemoglobinuric fever in Selangor, Malay Peninsula [duplicate2]

Watson M

J Trop Med, 1904;7:392-3.

Matched MeSH terms: Hemoglobinuria

Fulltext A case of paroxysmal nocturnal haemoglobinuria (PNH)

Koh CS

Med J Malaysia, 1987 Jun;42(2):122-3.

A 35 year old Chinese female with classical presentation of paroxysmal nocturnal haemoglobinuria is reported. The various aspects of these conditions are discussed.

Matched MeSH terms: Hemoglobinuria, Paroxysmal*

Fulltext Iptacopan monotherapy in patients with paroxysmal nocturnal hemoglobinuria: a 2-cohort open-label proof-of-concept study

Jang JH, Wong L, Ko BS, Yoon SS, Li K, Baltcheva I, et al.

Blood Adv, 2022 08 09;6(15):4450-4460.

PMID: 35561315 DOI: 10.1182/bloodadvances.2022006960

Iptacopan (LNP023) is a novel, oral selective inhibitor of complement factor B under clinical development for paroxysmal nocturnal hemoglobinuria (PNH). In this ongoing open-label phase 2 study, PNH patients with active hemolysis were randomized to receive single-agent iptacopan twice daily at a dose of either 25 mg for 4 weeks followed by 100 mg for up to 2 years (cohort 1) or 50 mg for 4 weeks followed by 200 mg for up to 2 years (cohort 2). At the time of interim analysis, of 13 PNH patients enrolled, all 12 evaluable for efficacy achieved the primary endpoint of reduction in serum lactate dehydrogenase (LDH) levels by ≥60% by week 12 compared with baseline; mean LDH levels dropped rapidly and durably, namely by 77% and 85% at week 2 and by 86% and 86% at week 12 in cohorts 1 and 2, respectively. Most patients achieved a clinically meaningful improvement in hemoglobin (Hb) levels, and all but 1 patient remained transfusion-free up to week 12. Other markers of hemolysis, including bilirubin, reticulocytes, and haptoglobin, showed consistent improvements. No thromboembolic events were reported, and iptacopan was well tolerated, with no severe or serious adverse events reported until the data cutoff. In addition to the previously reported beneficial effect of iptacopan add-on therapy to eculizumab, this study showed that iptacopan monotherapy in treatment-naïve PNH patients resulted in normalization of hemolytic markers and rapid transfusion-free improvement of Hb levels in most patients. This trial was registered at www.clinicaltrials.gov as #NCT03896152.

Matched MeSH terms: Hemoglobinuria, Paroxysmal*

Notes of a case of haemoglobinuric fever in Selangor, Malay Peninsula [duplicate1]

Watson M

Journal of the Malaya Branch of the British Medical Association, 1903.

Matched MeSH terms: Hemoglobinuria

Blackwater fever

Freer GD

Journal of the Malaya Branch of the British Medical Association, 1904.

Matched MeSH terms: Hemoglobinuria

Paroxysmal nocturnal haemoglobinuria: atypical presentation in a 13-year old Chinese and a Malay adult

Chu SF, Lopez CG, Duraisamy G, Eapen JS

Med J Malaysia, 1977 Dec;32(2):149-51.

PMID: 614482

Matched MeSH terms: Hemoglobinuria, Paroxysmal/diagnosis*

Fulltext Does glucose-6-phosphate dehydrogenase deficiency modify the course of leprosy or its treatment

Pettit JHS, Chin J

Lepr Rev, 1964 Jul;35(4):149-56.

PMID: 14177689

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.

Matched MeSH terms: Hemoglobinuria*

Fulltext Intravascular haemolysis with haemoglobinuria in a splenectomized patient with severe Plasmodium knowlesi malaria

Barber BE, Grigg MJ, William T, Yeo TW, Anstey NM

Malar J, 2016 Sep 09;15:462.

PMID: 27613607 DOI: 10.1186/s12936-016-1514-0

BACKGROUND: Haemoglobinuria is an uncommon complication of severe malaria, reflecting acute intravascular haemolysis and potentially leading to acute kidney injury. It can occur early in the course of infection as a consequence of a high parasite burden, or may occur following commencement of anti-malarial treatment. Treatment with quinine has been described as a risk factor; however the syndrome may also occur following treatment with intravenous artesunate. In Malaysia, Plasmodium knowlesi is the most common cause of severe malaria, often associated with high parasitaemia. Asplenic patients may be at additional increased risk of intravascular haemolysis.

CASE PRESENTATION: A 61 years old asplenic man was admitted to a tertiary referral hospital in Sabah, Malaysia, with severe knowlesi malaria characterized by hyperparasitaemia (7.9 %), jaundice, respiratory distress, metabolic acidosis, and acute kidney injury. He was commenced on intravenous artesunate, but1 day later developed haemoglobinuria, associated with a 22 % reduction in admission haemoglobin. Additional investigations, including a cell-free haemoglobin of 10.2 × 10(5) ng/mL and an undetectable haptoglobin, confirmed intravascular haemolysis. The patient continued on intravenous artesunate for a total of 48 h prior to substitution with artemether-lumefantrine, and made a good recovery with resolution of his haemoglobinuria and improvement of his kidney function by day 3.

CONCLUSIONS: An asplenic patient with hyperparasitaemic severe knowlesi malaria developed haemoglobinuria after treatment with intravenous artesunate. There are plausible mechanisms for increased haemolysis with hyperparasitaemia, and following both splenectomy and artesunate. Although in this case the patient made a rapid recovery, knowlesi malaria patients with this unusual complication should be closely monitored for potential deterioration.

Matched MeSH terms: Hemoglobinuria/chemically induced*; Hemoglobinuria/pathology

Assessing donor chimerism using flow cytometry in paroxysmal nocturnal haemoglobinuria after stem cell transplantation--a case report

Azma RZ, Hamidah NH, Leong CF, Ainoon O, Cheong SK

Malays J Pathol, 2006 Dec;28(2):107-12.

PMID: 18376800

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired haemopoietic stem cell disorder arising from somatic mutation of the X-linked PIG-A gene which leads to deficiency of the glycosylphosphatidylinositol (GP1) membrane anchor proteins such as CD 59 (MIRL: membrane inhibitor of reactive lysis) and CD 55 (DAF: decay accelerating factor). Allogeneic peripheral blood stem cell transplant (PBSCT) is a curative mode of treatment in symptomatic PNH patients. Assessment of donor chimerism for PBSCT can be performed by various methods including short tandem repeat loci (STR) and variable number of tandem repeats (VNTR). Flow cytometry, which is much cheaper and faster, also can be used to assess engraftment in patients with PNH. Engrafted patients will show the presence of CD 55 and CD 59 on their red cells and white cells. We describe here the usefulness of flow cytometry in the assessment of donor chimerism following allogeneic PBSCT, in a case of PNH.

Matched MeSH terms: Hemoglobinuria, Paroxysmal/blood; Hemoglobinuria, Paroxysmal/genetics; Hemoglobinuria, Paroxysmal/therapy*

Fulltext Ravulizumab (ALXN1210) vs eculizumab in adult patients with PNH naive to complement inhibitors: the 301 study

Lee JW, Sicre de Fontbrune F, Wong Lee Lee L, Pessoa V, Gualandro S, Füreder W, et al.

Blood, 2019 02 07;133(6):530-539.

PMID: 30510080 DOI: 10.1182/blood-2018-09-876136

Ravulizumab (ALXN1210), a new complement C5 inhibitor, provides immediate, complete, and sustained C5 inhibition. This phase 3, open-label study assessed the noninferiority of ravulizumab to eculizumab in complement inhibitor-naive adults with paroxysmal nocturnal hemoglobinuria (PNH). Patients with lactate dehydrogenase (LDH) ≥1.5 times the upper limit of normal and at least 1 PNH symptom were randomized 1:1 to receive ravulizumab or eculizumab for 183 days (N = 246). Coprimary efficacy end points were proportion of patients remaining transfusion-free and LDH normalization. Secondary end points were percent change from baseline in LDH, change from baseline in Functional Assessment of Chronic Illness Therapy (FACIT)-Fatigue score, proportion of patients with breakthrough hemolysis, stabilized hemoglobin, and change in serum free C5. Ravulizumab was noninferior to eculizumab for both coprimary and all key secondary end points (Pinf < .0001): transfusion avoidance (73.6% vs 66.1%; difference of 6.8% [95% confidence interval (CI), -4.66, 18.14]), LDH normalization (53.6% vs 49.4%; odds ratio, 1.19 [0.80, 1.77]), percent reduction in LDH (-76.8% vs -76.0%; difference [95% CI], -0.83% [-5.21, 3.56]), change in FACIT-Fatigue score (7.07 vs 6.40; difference [95% CI], 0.67 [-1.21, 2.55]), breakthrough hemolysis (4.0% vs 10.7%; difference [95% CI], -6.7% [-14.21, 0.18]), and stabilized hemoglobin (68.0% vs 64.5%; difference [95% CI], 2.9 [-8.80, 14.64]). The safety and tolerability of ravulizumab and eculizumab were similar; no meningococcal infections occurred. In conclusion, ravulizumab given every 8 weeks achieved noninferiority compared with eculizumab given every 2 weeks for all efficacy end points, with a similar safety profile. This trial was registered at www.clinicaltrials.gov as #NCT02946463.

Matched MeSH terms: Hemoglobinuria, Paroxysmal/drug therapy*; Hemoglobinuria, Paroxysmal/immunology; Hemoglobinuria, Paroxysmal/pathology

Blackwater fever or Lederer's anaemia?

Landor J

Journal of the Malaya Branch of the British Medical Association, 1937;1:184-87.

Matched MeSH terms: Hemoglobinuria

A bite by the Twin-Barred Tree Snake, Chrysopelea pelias (Linnaeus, 1758)

Ismail AK, Weinstein SA, Auliya M, Sabardin DM, Herbosa TJ, Saiboon IM, et al.

Clin Toxicol (Phila), 2010 Mar;48(3):222-6.

PMID: 20345298 DOI: 10.3109/15563650903550964

The Twin-Barred Tree Snake, Chrysopelea pelias, is a colubrine that, like other members of the genus Chrysopelea, is able to glide in the arboreal strata. Little is known about the effects of its bite. This report is the first clinically documented bite by this relatively uncommon rear-fanged species.

Matched MeSH terms: Hemoglobinuria/etiology

Successful treatment of acute kidney injury secondary to haeme nephropathy in paroxysmal nocturnal haemoglobinuria with alkaline diuresis

Sakthiswary R, Das S, Fadilah SA

EXCLI J, 2012;11:198-203.

PMID: 27298607

Paroxysmal nocturnal haemoglobinuria (PNH) also known as 'Marchiafava Micheli syndrome' is a rare condition which can lead to both acute and chronic forms of renal failure through renal tubular haemosiderin deposition. A 45-year-old lady with underlying PNH, presented with complaints of fever, productive cough followed by dark coloured urine. Investigations revealed pancytopenia with a markedly raised creatinine from her baseline (from 65 mmol/L to 385 mmol/L) consistent with acute kidney injury (AKI). Renal biopsy confirmed the diagnosis of haeme nephropathy. The renal impairment improved rapidly and normalised over a period of 5 days with alkaline diuresis (AD). The patient did not require haemodialysis unlike most other reported cases of AKI secondary to haeme nephropathy in PNH. This is the second reported case of AKI in PNH which was successfully treated with AD alone emphasizing the role of AD as a promising therapeutic strategy in this condition.

Matched MeSH terms: Hemoglobinuria, Paroxysmal

Fulltext Correlation of BACH1 and Hemoglobin E/Beta-Thalassemia Globin Expression

Lee TY, Muniandy L, Teh LK, Abdullah M, George E, Sathar J, et al.

Turk J Haematol, 2016 Mar 05;33(1):15-20.

PMID: 26377036 DOI: 10.4274/tjh.2014.0197

The diverse clinical phenotype of hemoglobin E (HbE)/β-thalassemia has not only confounded clinicians in matters of patient management but has also led scientists to investigate the complex mechanisms involved in maintaining the delicate red cell environment where, even with apparent similarities of α- and β-globin genotypes, the phenotype tells a different story. The BTB and CNC homology 1 (BACH1) protein is known to regulate α- and β-globin gene transcriptions during the terminal differentiation of erythroid cells. With the mutations involved in HbE/β-thalassemia disorder, we studied the role of BACH1 in compensating for the globin chain imbalance, albeit for fine-tuning purposes.

Matched MeSH terms: Hemoglobinuria/genetics; Hemoglobinuria/metabolism; Hemoglobinuria/epidemiology

Molecular basis of beta-thalassemia in the Maldives

Furuumi H, Firdous N, Inoue T, Ohta H, Winichagoon P, Fucharoen S, et al.

Hemoglobin, 1998 Mar;22(2):141-51.

PMID: 9576331

We have systematically analyzed beta-thalassemia genes using polymerase chain reaction-related techniques, dot-blot hybridization with oligonucleotide probes, allele specific-polymerase chain reaction, and sequencing of amplified DNA fragments from 41 unrelated patients, including 37 beta-thalassemia homozygotes, three with beta-thalassemia/Hb E, and one with beta-thalassemia/Hb S. Four different beta-thalassemia mutations were detected in 78 alleles. These are the IVS-I-5 (G-->C), codon 30 (AGG-->ACG) [also indicated as IVS-I (-1)], IVS-I-1 (G-->A), and codons 41/42 (-TTCT) mutations. The distribution of the beta-thalassemia mutations in the Maldives is 58 alleles (74.3%) with the IVS-I-5 (G-->C) mutation, 12 (15.4%) with the codon 30 (AGG-->ACG) mutation, seven (9%) with the IVS-I-1 (G-->A) mutation, and one with the codons 41/42 (-TTCT) mutation. The first three mutations account for 98.7% of the total number of beta-thalassemia chromosomes studied. These mutations are clustered in the region spanning 6 bp around the junction of exon 1 and the first intervening sequence of the beta-globin gene. These observations have significant implications for setting up a thalassemia prevention and control program in the Maldives. Analysis of haplotypes and frameworks of chromosomes bearing each beta-thalassemia mutation suggested that the origin and spread of these mutations were reflected by the historical record.

Matched MeSH terms: Hemoglobinuria/complications; Hemoglobinuria/genetics

Fulltext Glycaemic control of diabetic patients in an urban primary health care setting in Sarawak: the Tanah Puteh Health Centre experience

Wong JS, Rahimah N

Med J Malaysia, 2004 Aug;59(3):411-7.

PMID: 15727390 MyJurnal

Achieving glycaemic goals in diabetics has always been a problem, especially in a developing country with inadequate facilities such as in Sarawak in Malaysia. There are no reported studies on the control of diabetes mellitus in a diabetic clinic in the primary health care setting in Sarawak. This paper describes the profile of 1031 patients treated in Klinik Kesihatan Tanah Puteh Health Centre. The mean age was 59 years, the mean BMI 27 kg/m2. There was a female preponderance and mainly type-2 diabetes. Mean HbA1c was 7.4%. Glycaemic control was optimal in 28% (HbA1c <6.5%), fair in 34% (HbA1c 6.5-7.5%) and poor in 38% (HbA1c >7.5%). Reasonable glycaemic control can be achieved in the primary health care setting in Sarawak.
Study site: Klinik Kesihatan Tanah Puteh, Sarawak, Malaysia

Matched MeSH terms: Hemoglobinuria/metabolism

A preliminary evaluation of the efficacy and safety of Cogent db (an ayurvedic drug) in the glycemic control of patients with type 2-diabetes

Shekhar KC, Achike FI, Kaur G, Kumar P, Hashim R

J Altern Complement Med, 2002 Aug;8(4):445-57.

PMID: 12230905

A nonrandomized, non-placebo-controlled clinical trial to evaluate the efficacy of Cogent db (an herbal preparation; Cybele Herbal Laboratories [PVT] Ltd. Kochi, Kerala State, India) as an adjuvant in the treatment of patients with type 2 diabetes was carried out during a 3-month period.

Matched MeSH terms: Hemoglobinuria

The principles of snakebite treatment

Reid HA

Clin. Toxicol., 1970 Sep;3(3):473-82.

PMID: 5520050

Matched MeSH terms: Hemoglobinuria

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