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Coagulopathy usually presents as soft tissue bleeding, seldom are they seen
as spontaneous gastrointestinal or intraperitoneal bleeding.
The study present a case of a Filipino with acquired factor X deficiency
secondary to a plasma cell disorder presenting as upper gastrointestinal
bleeding.
Case Report
A 52 year-old Filipino, male, was previously well until February 2009 when he
presented with atraumatic acute surgical abdomen
He underwent emergency exploratory laparotomy with evacuation of hematoma and
massive hemoperitoneum
Due to difficulty of surgical hemostasis, bleeding parameters were requested. It
showed prolonged prothrombin time (PT) and activated partial thromboplastin time
(APTT), platelet count was normal.
Fresh frozen plasma was transfused and vitamin K was administered which controlled
the bleeding.
There was no recurrence of bleeding, but bleeding parameters were persistently
abnormal, he was discharged with vitamin K tablet. No work-up was done.
He was asymptomatic for three months until November 2009 when he had an episode
of massive hematochezia and abdominal pain, hence admission at the institution.
Case Report
This patient satisfied the three minor criteria for multiple myeloma namely
presence of lytic bone lesions, monoclonal lambda light chains on urine
electrophoresis and immunofixation and plasma cells of 14%.
The multisystem involvement led us to consider primary systemic amyloidosis
and so a fat pad biopsy was sent for congo red stain which turned out to be
negative for amyloid component and negative for malignant cells.
Case Report
The plan for this patient was to establish the presence of amyloidosis by
biopsy of involved critical organs because it has a higher sensitivity and
specificity at 90-100%.1 It is difficult in this case because it carries an 8% risk
of suffering from procedure related hemorrhage and the patient did not give
consent.
Treatment plan is oral chemotherapy with melphalan and prednisone for the
multiple myeloma cannot totally rule out primary systemic amyloidosis.
DISCUSSION
Multiple myeloma was confirmed by the presence of three minor criteria but
due to multiorgan involvement and the fact that 10-15% of multiple
myelomas are associated with primary systemic AL-amyloidosis, it was also
considered in this patient.
Tissue diagnosis of amyloidosis should be confirmed by biopsy of the
abdominal fat which turned out to be negative in our patient or biopsy of
critical organs which is high risk for hemorrhagic complications.
The mainstay of treatment for acute bleeding due to factor X deficiency
consist of fresh frozen plasma (FFP) with a loading dose of 15-20 ml/ kg
followed by 3-6 ml/kg twice daily to keep factor X levels above 10-20%.
Other alternatives are pro-thrombin complex concentrate (PCC) and factor X
concentrates but are unavailable in many developing countries.
CONCLUSION