an unusual case of a bleeding disorder : dr m khan
TRANSCRIPT
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An unusual case of a bleeding disorder.
By: Dr M Khan
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Index caseMr KS 18 years from Imbali in EdendalePietermaritzburg.Presenting complaint : Epistaxis – 3 days , intermittent , both nostrils , no preceding trauma . 1st episode
plus Haematuria – macroscopic with clots ,1 day , intermittent , no BOM , no FOM , no fever
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Systemic enquiryMusculoskeletal : no joint pain / swelling
GIT : no malaena / haematochezia / haematemasis
CNS : No focal weakness / severe headache
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Past medical historyNever admitted to hospital
No bleeding disorder
Not on any traditional / OTC medication
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Social history
Grade 11 scholar – passing with good grades
Cared by father ( shop assistant )
Access to electricity and clean water
Sober habits , non smoker
Never exposed to rats at home / school
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Family historyNo history of bleeding problems
No family members on anticoagulants
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On examinationWell looking apyrexialMinimal bleeding from both nostrilsDipstick : 3 + blood , no leucocytesNo : pallor / koilonychia / angular stomatitis / orocutaneous bleeding / stigmata of immunocompromize
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CVS : pulse 90 , BP 110 / 80 , not in failure , no features of infective endocarditis .
Resp : not distressed / clear
Abd :Not in liver failure / No features of chronic liver disease - SNT ,PR – no malaena
CNS : no focal deficit , no meningism
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AssessmentBleeding disorder with epistaxis and haematuria
Differential diagnoses : 1. Haemophilia Spectrum2. DIC3. Overdose ( accidental / intentional )
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ManagementAdmit
Urgent bloods including clotting profile and FBC
Urine MCS
Nasal packing
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ResultsFBC : Hb 11.7 ( MCV 89 / MCH 30 ) Platelets 211 ( MPV 8.8 ) WCC8.5
LFT : TP 73 , Alb 41 , Tbil 10 , ALP 48 , GGT 26 , ALT 12 , LDH 454
U&E : Na 137 , K 4 , Cl 105 , CO2 23 , Urea 3.2 , Creatinine 66
INR 12.5 , APTT 68.1 ( 24.1 ) , Fibrinogen normal
Urine MCS : RBC’s > 100 / no growth / no ova
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Vitamin K 10 mg ivi stat and daily
Haemodynamic monitoring
Repeat clotting profile
Started on antibiotic prophylactically
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That night Registrar called : patient developed sublingual mucosal haematoma which was rapidly increasing in size ? Impending airway obstructing
FBC : Hb 7.2 / platelets 184 / WCC 6.63
INR 8.1 / APTT 86.4 ( 26.4 )
Management : Transfused 2 units blood , 2 units fresh frozen plasma and monitored closely
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ReviewedSwelling static – not increasing in size
Hb 8.4 / INR 2.52 / APTT 38.3 ( 26.4 )
Mucosal swelling decreased significantly by the next morning
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?? Diagnosis ??
prolongedprolongedprolongedprolongedAPTT
prolongedGrossly prolonged
normalnormalINR
lownormalNormalNormalPlatelet count
Liver diseaseOral Anticoagulants
Haemophilia BHaemophilia A
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Thus most likely diagnosis oral anticoagulant toxity
However patient has never been on warfarin& has no family members / contacts on warfarin
Vitamin K dependant factor assay done
Discharged to be reviewed in 3 /7
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Reviewedno bleedingfactor 2 5.1 %factor 7 0.7 % normal = 50 – 150%factor 9 1.6 %factor 10 19.5 %INR > 10APTT 125.2
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SUPERWARFARIN POISENINGCoumarins such as warfarin are vitamin K antagonists – oral anticoagulants & rodenticides inhibit the enzyme vitamin K epoxide reductaseaccumulation of vit K epoxide ( an inactive form ) causing an apparent vit K deficiency
Warfarin resistance in rats superwarfarins = rodenticides – extremely long acting and fat soluble and are 100 times more potent than warfarin
Brodifacoum most commonly used superwarfarin ( in OTC rat poisens ) thus easily available accidental or delibrate ingestion not uncommon
Superwarfarin toxity cannot be easily reversed with standard doses of vitamin K .
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Poovalingum , Kenoyer , Bassa et al SAMJ November 2002 – 4 cases of superwarfarin poisening
CASE 1- 26 yr female assaulted by boyfriend following disclosure of hiv status- Multiple blows to lower abdomen lap performed to drain pelvic
haematoma- Started to bleed from operative site plus conjuctival haemorrhages- Vitamin K dependant factors low ( factor 5 and 8 were normal ) bleeding
stopped after administration of fresh frozen plasma corrrection of INR and APTT after addition of normal plasma
- Diagnosis of vit K deficiency was clearly confirmed but lack of sustained response to vit K pointed to super warfarin toxity
- Vit K1 100mg/ day ( 2 months ) but still required intermittent plasma infusions continued on oral vitamin K
- Eventually admitted to consuming Rattex – OTC rodenticide ( defethiolone )
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CASE 214 year old street child who regularly scavenged for food in rubbish bins –epistaxis and regular bleeding from venepuncture sitesBlood profile in keeping with vit K deficiencyNo hepatic / renal impairmentReceived vit K 100 mg iv in 3 divided doses and fresh frozen plasma as needed to control bleedingIn spite of treatment with oral and iv vit K he continued to bleed intermittentlyPhenobarbital added – no responseAbsconded after 3 ½ months of hospitalizationDied mysteriously – most likely due to coagulopathy
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CASE 3- 38 year old man who used Rattex regularly to sprinkle floors
because of a major rat infestation- Bleeding gums , frank haematuria , and abdominal pain- Lab tests – in keeping with vit K deficiency with lack of response to
treatment superwarfarin poisening- Treated like other patients- Stabilized on high doses of oral vit K1 - At 6 months follow up – well – still had lab evidence of a
coagulopathy
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CASE 453 year old man treated successfully for renal calculi at a local hospital1 week later – haematuria , bleeding from venepuncturesites and gum bleedingResults in keeping with vit K deficiencyRequired high doses of iv vit K1 Was returned to care of GP who continued daily iv injections until 8 months then swiched to oral vit K1Denied self poisening but did have family problemsCollateral info from GP revealed that he had 1 previous episode of overdose on antidepressants.
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50-150124.227Fac 1050-1502112.281.2Fac 950-15032.411Fac 750 -15011-93.7Fac 250-1501161538764Fac 51.5 -4.56.435.936.85.12Fibringen29-4462.483.381.6120APTT11-1466.691.2128110PT
NormalCase 4Case 3Case 2Case 1Test
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DISCUSSIONThus superwarfarin poisening is growing problem .Suicide attempts , industrial exposure or accidental poiseningPresents as a bleeding disorder Diagnosis : prolonged INR and APTT , normal fibrinogen and platelet count (excludes DIC ). Addition of normal plasma in the 50 /50 test – correction of prolonged coagulation times confirming specific factor deficiencies rather than the presence of an inhibitor . VitK dependant factor assays are low while factors 5 , 8 and fibrinogen are normal
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Thus lack of a sustained response to initial treatment with vit K and failure of resolution of the bleeding diathesis suggests superwarfarinexposure . The presence of superwarfarin in the blood or other body tissues confirms the diagnosis but these assays are only available in specialized centres .Treatment : -high doses of vit K1 – initially iv administration is often necessary – given as slow iv injection diluted with saline or glucose and can be repeated every 6 hours . Fresh frozen plasma is reserved for severe bleeding and red cell transfusions if blood loss is excessiveSuperwarfarins have long ½ lives and are fat soluble- treatment has to be continued for months and even yearsInconclusive evidence that phenobarbital enhances hepatic microsomal enzymes to increase metabolism of 4 hydroxycoumarinderivatives was not found to be useful in durban experience.
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Follow up my caseAdmitted to King Edward haematologydepartmentReceived intravenous vitamin K1 plus intermittent fresh frozen plasma intermittently for 2 weeksDischarged on oral vitamin K1 to be followed up at Edendale