title: von Willebrand Disease : A Clinico-haematological Spectrum.
abstract: BACKGROUND
  Bleeding disorders are commonly seen in clinical practice. von Willebrand Disease (vWD), is the commonest and yet a profoundly under diagnosed cause, having a wide spectrum of clinical presentation. Of its three types, type 1 vWD (70% of the total vWD cases) has the mildest and a highly variable clinical and laboratory presentation.
  
  
  METHODS
  A series of ten cases of vWD were comprehensively evaluated using recommended diagnostic parameters and therapeutic interventions.
  
  
  RESULTS
  All major types of vWD were represented. A female preponderance, with primary presentation in the form of muco-cutaneous bleeds was observed. A positive history of consanguineous parental marriage and family history of bleeding disorder was elicited in two and three patients respectively. Nine patients were found to be anemic and thrombocytopenia was present in only one. Bleeding time by modified template (SIMPLATE) method, along with activated partial thromboplastin time (APTT) was increased in all ten cases and of these, nine had low factor VIII: C levels. Ristocetin induced platelet aggregation studies were abnormal in all the five cases it was performed. vWF:RCo activity determined in one individual was shown to be low. vWF:Ag assay was done in four cases revealing a near complete absence of von Willebrand factor antigen in one and mildly decreased levels in the other three. vWF multimer assay was advised in three cases. DDAVP, plasma derived vWF, blood products and local antifibrinolytics were used as primary modalities of treatment.
  
  
  CONCLUSIONS
  Thus, strong clinical suspicion, thorough clinical evaluation and judicious use of investigations including repeated investigations at different times are needed for making a diagnosis of vWD.
authors.affiliation: Classified Specialist (Pathology and Haematology), Army Hospital (R and R), Delhi Cantt.
authors.forename: D K
authors.initials: DK
authors.lastname: Mishra
authors.affiliation: Post Graduate Resident, Department of Pathology, Armed Forces Medcial College, Pune 411 040.
authors.forename: A
authors.initials: A
authors.lastname: Chaturvedi
authors.affiliation: Classified Specialist (Medicine and Haematology), Army Hospital (R and R), Delhi Cantt.
authors.forename: A
authors.initials: A
authors.lastname: Sharma
authors.affiliation: PMO, HQ Eastern Air Command, C/o 99 APO.
authors.forename: H
authors.initials: H
authors.lastname: Subramanya
authors.affiliation: Associate Professor, Department of Blood Transfusion, Armed Forces Medcial College, Pune 411 040.
authors.forename: Harsh
authors.initials: H
authors.lastname: Kumar
authors.affiliation: Classified Specialist (Medicine and Neurology), Command Hospital (Air Force), Bangalore.
authors.forename: R
authors.initials: R
authors.lastname: Varadarajulu
authors.affiliation: Senior Advisor (Medicine), Command Hospital (Eastern Command), Calcutta.
authors.forename: K P
authors.initials: KP
authors.lastname: Anand
keywords: Bleeding disorders; von Willebrand Disease