For mail.pmd


9

Introduction
Disseminated intravascular coagulation (DIC) is a
syndrome characterized by inappropriate and excessive
activation of haemostatic system. DIC is usually initiated
by exposure of blood to tissue factor, presents on the cell
surface that surround blood vessels. Brain and placenta
are especially rich in tissue factor1. DIC results from
trauma, obstetric accident, diffuse vascular injury,
increased endothelial permeability or circulating cancer
cells resulting pathologic activation of the extrinsic and/
or intrinsic pathway of coagulation or impairment of clot
inhibiting influences2,3. In subacute or chronic DIC, there
is slow activation of haemostatic system, with spontaneous
bruising rather than major clinical bleeding episodes.
Rarely, a chronic compensated form of DIC can continue
for many years, usually associated with intrauterine
infection, internal malignancy or vascular malformations.
The principles of management of DIC are control of
haemorrhage, elimination of precipitating factors and
specific coagulation factor replacement therapy. Here we
are going to present such a rare case of chronic DIC.

Case report
A 35-years-old woman was admitted in Medicine
department of Bangabandhu Sheikh Mujib Medical
University (BSMMU) on 11th March 2004 with the
complaints of echymoses of variable sizes and color for 5
months. She complained of epistaxis, gum bleeding,
haematuria, melaena and menorrhagia. She gave history
of prolonged bleeding after minor trauma and delayed

Chronic Disseminated Intravascular Coagulation: A Case Report
Md. Abul Kalam Azad1, M Abdul Kader2, M Abdul Jalil Chowdhury3, Tofayel Ahmed3
1Associate Professor of Medicine, Bangabandhu Sheikh Mujib Medical University, 2Assistant Professor of Medicine, Bangabandhu Sheikh Mujib
Medical University, 3Professor of Medicine, Bangabandhu Sheikh Mujib Medical University.

Abstract:
In health there is a balance between the coagulation and anti-coagulation systems, but in disseminated intravascular
coagulation (DIC) the coagulation mechanism is activated inappropriately and in a diffuse way. This may lead to
thrombosis, but more often haemorrhage occurs when the clotting factors are exhausted. DIC may present as acute,
subacute, and rarely chronic form. Here we present a case of chronic DIC following pelvic inflammatory disease (PID)
as a consequence of repeated menstruation regulation (MR). We treated her with fresh frozen plasma, fresh blood,
doxycycline with significant clinical improvement.

[BSMMU J 2008; 1(1): 33-34]

wound healing for the said duration. She stated about
repeated oral ulcers without any fever, arthralgia/ arthritis,
bone pain, photosensitivity or malar rash. She had no
history of such previous bleeding episode either in her or
in her family members. She was mother of two healthy
children. She gave a history of MR seven months back,
but she remained amenorrhic for more than two months.
Her urinary HCG test was positive at that time, and
sonogrphic examination revealed retained product of
conception and Dilatation and curettage (D&C) was done.
She had no significant drug history except oral
contraceptive pill. On general examination, she was
anxious, moderately anemic, with multiple non-tender,
non-palpable echymoses on the extensor surface of both
limbs, of variable sizes and colours, which didn’t blanch
on pressure.

Her peripheral blood film was suggestive of anemia of
chronic disorder (Haemoglobulin 7.8gm/dl) with high
ESR (98mm in 1st hour) and normal platelet count
(155,000/uL). Sonogaphic examination of pelvic organ
revealed that uterus was mildly enlarged. Clotting time
was prolonged i.e. 18 minutes, with normal bleeding time.
Her prothrombin time was 48 second (control: 13 sec),
activated partial thromboplastin time (APTT) was 128
seconds (normal 30-40 seconds), thrombin time was 22
seconds (control: 16 sec) and factor VIII activity was
25% (normal range 60-150%). Her ANA was positive in
low titer (two fold rise) and anti-dsDNA and anti-Sm
test were negative. Initially she was suspected of having
vasculitic disorders and treated with steroid, but there was
no clinical improvement.  Her plasma fibrinogen was
2.37g/l (normal 1.5-4.0gm/l) but fibrin degradation

Correspondence to : Dr. Md. Abul Kalam Azad, Associate Professor
of Medicine, Bangabandhu Sheikh Mujib Medical University, Dhaka,
Bangladesh, E-mail: azadbsmmu@yahoo.com

CASE REPORTS



10

product (FDP) and D-dimer (normal ‹0.2mg/dl) were 5
µgm /l and 0.5 µgm/ ml respectively, i.e., both were
increased. Per vaginal examination revealed that uterus
was bulky with fluid collection in the pouch of Douglas
and diagnosed a case of chronic DIC due to pelvic
inflammatory disease (PID) as a consequence of retained
products of conception. She was treated with cap.
Doxycycline (100 mg) b.d. for one month as well as with
five units of fresh frozen plasma and one unit of whole
fresh blood with significant clinical improvement. After a
few days, her coagulation profile was repeated and was
within normal limit (PT -14 seconds, APTT -38 seconds,
TT -12 seconds, plasma fibrinogen 250 mg/dl, FDP-<05
µgm /ml and D-dimer-<0.5 µgm /ml).

Discussion:
Disseminated intravascular coagulation is an acute,
subacute or chronic thrombohaemorrhagic disorder
occurring as a complication in a variety of diseases. It is
characterized by activation of the coagulation sequence
that leads to the formation of microthrombi throughout
the microcirculation of the body and accelerated
fibrinolysis4. 

DIC usually presents as an acute, often catastrophic,
acquired haemorrhagic tendency. Rarely it can also
manifest as a low grade disorder with predominantly
thrombotic manifestations5. Chronic DIC may occur due
to intrauterine fetal death, giant haemangiomas (Kasabach
Merritt syndrome) or adenocarcinoma. Intrauterine
infection causes endotoxins to be released into the maternal
circulation and damage of the blood vessels releases
thromboplastins, which causes chronic DIC. Other
etiologic factors of chronic DIC are aneurysms, vasculitis,
leiomyomas, hydatidiform mole etc6. In chronic DIC,
intravascular coagulation and fibrinolysis don’t proceed
fast enough to outstrip the rate of synthesis of clotting
factors or inhibitors. This may simply reflect low grade,
weak or intermittent activating stimulus, in which case
DIC is often mild and asymptomatic7. Destruction and
production of coagulation factors and platelets are
balanced. The pathophysiology of such chronic, subacute
or compensated DIC is fundamentally the same as that in
the acute case. Nevertheless, the distinction is valuable
because the clinical pictures and laboratory findings in
the chronic form are quite variable and may be
diagnostically confusing6.

In chronic DIC, superficial but extensive ecchymosis of
extremities may develop intermittently or may persist for
weeks or months. Recurrent episodes of epistaxis or more

serious internal mucosal bleeding may punctuate the
course. DIC, caused by carcinoma, may cause bleeding
or recurrent deep and superficial venous thrombosis8.
Intrauterine fetal death may produce chronic DIC,
particularly if the fetus is retained for several weeks9. In
chronic or subacute DIC, prothrombin time, activated
partial thromboplastin time and thrombin time are
prolonged but platelet count may be normal or low.
Fibrinogen concentration may be normal or low. However,
there is usually an increase in fibrin degradation product
(FDP) and increased level of D-dimer5.

Conclusion:
Chronic or subacute DIC is a rare, catastrophic
haemorrhagic disorder or sometimes shows a thrombo-
embolic manifestation. We diagnosed her as a case of
chronic DIC due to retained product of conception. But
we failed to explain why her ANA was increased. For this
reason she should further followed up as she may develop
any connective tissue disease. It is further to see whether
ANA disappear spontaneously.

References
1. Drake T, Morrissey J, Edgington T. Selective cellular expression

of tissue expression factor in humans tissues. Implications for
disorders of haemostasis and thrombosis. Am J Pathol 1989; 134:
1087-2001.

2. Lijima K, Fukuda C, Nakamura K. Measurements of tissue factor
like activity in plasma of patients with DIC. Throb Res 1991;
61:29-41.

3. Cortan RS, Kumar V, Robbins LS. Robins Pathologic basis of
disease. 6th ed. Bangalore: W.B. Saunders Company;  1999. p
623-626.

4. Machin JS. Acquired Coagulation disorder. In; Hoffbrand AV,
Lewis SM, Tuddenham ED, editors. Postgraduate haematology.
Oxford: Butterworth Heinemann; 1999. p.640-645.

5. Brozovic M. Acquired disorders of blood coagulation. In: Bloom
AL and Thomas DP, editors. Haemostasis and thrombosis. New
York; Churchill Livingstone; 1981;  p.640-645.

6. Grosset AB, Rodgers GM. Acquired coagulation disorders. In:
Richard D, Foster J, Rodgers GM, editors. Wintobe’s clinical
haematology. Baltimore: Wilkin’s; 1999; p.1733-1753.

7. Williams EC, Mosher DH. Disseminated intravascular
coagulation. In: Hoffmann R, Shattil SJ, Edward JB, editors. Basic
principles and practice of hematology. New York: Churchill
Livingstone; 1995; p. 1758-1766.

8. Sack J, Levin J, Bell W. trosseau’s syndrome and other
manifestations of chronic disseminated coagulopathy in patients
with neoplasms. Medicine 1977;  56: 1-8.

9. Hatch RL, Barke JI, Barke MW. Coagulopathy associated with
dilatation and evacuation for intrauterine fetal death. Obst and
Gynaecol 1985;  66: 463-478.

BSMMU J Vol. 1, Issue. 1, July 2008

34