A Specific Coagulation Disorder of Uncommon Cause


Abstract

BACKGROUND: Coagulation abnormalities are common in clinical practice. Causes  include: 

1. Vitamin K deficiency due to a) liver and gall bladder diseases, b) cystic fibrosis, c) intestinal  (malabsorption or intake deficiency), d) medications and toxins, e) Vitamin K dependent clotting factor deficiency (VKCFD) (genetic). 2, Antibodies against clotting factors, e.g. factor VIII 

3. Disseminated intravascular coagulation. 

CASE DESCRIPTION :61-year-old female with hypertension presented with haematuria-1  day. No dysuria. History of epistaxis and melena 2 and 3 days back. No history suggestive of  malabsorption or liver/gallbladder disease. No history of any trauma, intake  of herbal/alternative medicines or special diet. No past, obstetric and family history  significant of bleeding. 

On examination, conscious and oriented. Vital signs were stable. Systemic  examination: Normal. Laboratory investigations showed hemoglobin of 11.9g/dl. Clotting  time (29min), prothrombin time (INR 13.3), activated partial thromboplastin (aPTT) (96.9 sec)  were elevated. Mixing study showed correction of prothrombin time and activated  partial thromboplastin time (aPTT) by normal plasma indicating factor deficiency. Factor  assay showed deficiency of vitamin K dependent coagulation factors (II, VII, IX, X). Other  factors were normal. Patient was treated with fresh frozen plasma, cryoprecipitate,  vitamin K causing partial correction of the coagulation factors. Her bleeding symptoms  resolved and is on follow up. INR and aPTT are still out of range. 

CONCLUSION: Common causes of vitamin K deficiency were not present  after detailed history, physical examination and investigations. Treatment with vitamin K  improved the symptoms but was not able to correct the laboratory findings. A mild genetic defect of VKCFD which was asymptomatic and manifested now at a later stage  is also considered. She is now on vitamin K and on follow up 

and clotting factor assay will be done to determine the cause. • Complete correction of factors will suggest deficiency of vitamin K due to obscure  acquired causes. 

• Partial correction of clotting factors will suggest a mild form of rare hereditary  VKCFD. We also plan to do a genetic test in the future (if possible).

 

Poster
non-peer-reviewed

A Specific Coagulation Disorder of Uncommon Cause


Author Information

Hridya Harimohan Corresponding Author

Internal Medicine, Kerala Institute of Medical Sciences, Trivandrum, IND

Mathew Thomas

Department of Internal Medicine and Hematology, Kerala Institute of Medical Sciences, Trivandrum, IND

Neeraj Sidharthan

Hematology and Medical Oncology, Amritha Institute of Medical sciences, Thrissur, IND


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