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A Rare Case of von Willebrand Disease Presenting as Hemolacria and Literature Review

Nandakumal G, Ismail F, Mohamad NF, Lott PW, Chew KS, Ab Rahman S, et al.

J Pediatr Hematol Oncol, 2021 04 01;43(3):101-103.
PMID: 33560075 DOI: 10.1097/MPH.0000000000002077

Hemolacria is a rare condition that causes a person to produce tears that are partially composed of blood. It can be a presenting feature of certain ocular and systemic conditions. Here, the authors describe an interesting case of a 12-year-old boy with an underlying beta-thalassemia trait, who presented with a 2-day history of bilateral blood-stained tears, and an episode of epistaxis. Ocular examination was normal, and syringing showed no nasolacrimal duct blockage. Systemic examination was unremarkable. Laboratory investigations confirmed type 2 von Willebrand disease. Management of hemolacria remains a clinical challenge given the rare occurrence of the disease. In this case report, the authors discuss the differential diagnosis and management approach to hemolacria.

Matched MeSH terms: von Willebrand Diseases/blood; von Willebrand Diseases/complications; von Willebrand Diseases/diagnosis*
Fulltext Conventional rapid latex agglutination in estimation of von Willebrand factor: method revisited and potential clinical applications

Mahat M, Abdullah WZ, Hussin CM

J Immunol Res, 2014;2014:850810.
PMID: 25759835 DOI: 10.1155/2014/850810

Measurement of von Willebrand factor antigen (VWF : Ag) levels is usually performed in a specialised laboratory which limits its application in routine clinical practice. So far, no commercial rapid test kit is available for VWF : Ag estimation. This paper discusses the technical aspect of latex agglutination method which was established to suit the purpose of estimating von Willebrand factor (VWF) levels in the plasma sample. The latex agglutination test can be performed qualitatively and semiquantitatively. Reproducibility, stability, linearity, limit of detection, interference, and method comparison studies were conducted to evaluate the performance of this test. Semiquantitative latex agglutination test was strongly correlated with the reference immunoturbidimetric assay (Spearman's rho = 0.946, P < 0.001, n = 132). A substantial agreement (κ = 0.77) was found between qualitative latex agglutination test and the reference assay. Using the scoring system for the rapid latex test, no agglutination is with 0% VWF : Ag (control negative), 1+ reaction is equivalent to <20% VWF : Ag, and 4+ reaction indicates >150% VWF : Ag (when comparing with immunoturbidimetric assay). The findings from evaluation studies suggest that latex agglutination method is suitable to be used as a rapid test kit for the estimation of VWF : Ag levels in various clinical conditions associated with high levels and low levels of VWF : Ag.

Matched MeSH terms: von Willebrand Diseases/diagnosis*
Effect of the Novel Biodegradable N, O-Carboxymethylchitosan and Oligo-Chitosan on the Platelet Thrombogenicity Cascade in von Willebrand Disease

Periayah MH, Halim AS, Mat Saad AZ, Yaacob NS, Hussein AR, Abdul Karim F, et al.

Thromb Res, 2015 Sep;136(3):625-33.
PMID: 26254703 DOI: 10.1016/j.thromres.2015.07.027

Von Willebrand disease (vWD) is the second least common hemostatic disorder in Malaysia, and it has a low prevalence. This study examined the underlying platelet thrombogenicity cascades in the presence of different formulations of chitosan-derivatives in vWD patients. This paper aimed to determine the significant influence of chitosan biomaterial in stimulating the platelet thrombogenicity cascades that involve the von Willebrand factor, Factor 8, Thromboxane A2, P2Y12 and Glycoprotein IIb/IIIa in vWD.

Matched MeSH terms: von Willebrand Diseases/blood*; von Willebrand Diseases/drug therapy*
Fulltext Report on von Willebrand Disease in Malaysia

Periayah MH, Halim AS, Saad AZ, Yaacob NS, Karim FA

Open Access Maced J Med Sci, 2016 Mar 15;4(1):112-7.
PMID: 27275342 DOI: 10.3889/oamjms.2016.030

BACKGROUND: Von Willebrand disease (vWD) is an inherited hemostatic disorder that affects the hemostasis pathway. The worldwide prevalence of vWD is estimated to be 1% of the general population but only 0.002% in Malaysia.
AIM: Our present paper has been written to disclose the statistical counts on the number of vWD cases reported from 2011 to 2013.
MATERIAL AND METHODS: This article is based on sociodemographic data, diagnoses and laboratory findings of vWD in Malaysia. A total of 92 patients were reported to have vWD in Malaysia from 2011 to 2013.
RESULTS: Sociodemographic-analysis revealed that 60% were females, 63% were of the Malay ethnicity, 41.3% were in the 19-44 year old age group and 15.2% were from Sabah, with the East region having the highest registered number of vWD cases. In Malaysia, most patients are predominately affected by vWD type 1 (77.2%). Factor 8, von Willebrand factor: Antigen and vWF: Collagen-Binding was the strongest determinants in the laboratory profiles of vWD.
CONCLUSION: This report has been done with great interest to provide an immense contribution from Malaysia, by revealing the statistical counts on vWD from 2011-2013.

Matched MeSH terms: von Willebrand Diseases
Fulltext Puerperal acquired factor VIII inhibitor causing a von Willebrand-like syndrome in a patient with anti-DNA antibodies

Jackson N, Hashim ZA, Zainal NA, Jamaluddin N

Singapore Med J, 1995 Apr;36(2):230-1.
PMID: 7676276

A 30-year-old Malay lady, with no previous or family history of bleeding, presented with severe gum bleeding 25 days post-partum. The factor VIII:c was 0.03 iu/ml with evidence of a slow-acting factor VIII inhibitor. Von Willebrand factor antigen (VWF:age) varied from less that 0.05 to 0.17 iu/ml, and there was absent ristocetin-induced platelet aggregation. Anti-nuclear and anti-DNA antibodies were present, but there were no other features of systemic lupus erythematosus. There was some clinical response to cryoprecipitate and tranexamic acid, and slight improvement with corticosteroid. Fifteen months later, the patient has no active bleeding problem, and her VWF-ag is increasing spontaneously. However, factor VIII:c is less than 0.01 iu/ml and her factor VIII inhibitor titre is still > 20 Bethesda units/ml.

Matched MeSH terms: von Willebrand Diseases/etiology*
Congenital coagulation disorders in Malaysia

Duraisamy, G.

Malaysian Journal of Child Health, 1998;10(1):62-71.
MyJurnal

Congenital Coagulation Disorders (CCD) are inherited and present from birth. Their diagnosis depends on clinical awareness and correct laboratory investigations. The central registry for CCD or Congenital Bleeding Disorders (CBD) is at the Blood Services Centre, Kuala Lumpur Hospital and was established in 1975. There are 871 CCD registered. The commonest CCD are 631 (72%) Haemophilia A, 102 (12%) Haemophilia B and 93 (10.7%) von Willebrand's Disease. The other deficiencies registered are rare, only 45 in total:— Factor 1 (4), FV (4), FVII (21), FX (4), FXII (6), and FXIII (6). Diagnosis is based on clinical suspicion, screening tests namely the Prothrombin Time (PT) and activated Partial Thromboplastin Time (aPTT) and confirmation of the diagnosis was by doing specific factor assays. Molecular studies were done on FVIII and FXIII. Treatment is by transfusing the deficient factor when there is bleeding and comprehensive care involving the specialities like the neurologist/ neurosurgeon /orthopaedic / physiotherapy/ dental besides the haematologist and paediatrician to manage the complicatioons seen. There are fewer problems now as patients are diagnosed earlier and managed better. There is now a good prognosis and a better quality of life.

Matched MeSH terms: von Willebrand Diseases