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|Year : 1976 | Volume
| Issue : 3 | Page : 157-159
Hypercoagulable state in diguglielmo's syndrome
Pradeep Mathur, VP Singh, B Dube, HS Bajpai
Institute of Medical Sciences, Banaras Hindu University, Varanasi-5, India
Institute of Medical Sciences, Banaras Hindu University, Varanasi-5
Source of Support: None, Conflict of Interest: None
The case history of a 28 year old lady with diGuglielmo's syndrome has been described. On admission, she had mixed myeloid and erythroid proliferation, but soon a picture of frank acute myeloblastic leukemia developed. The blood coagulation and fibrinolytic studies at admission revealed "Hypercoagulable State with insipient defibrination.
|How to cite this article:|
Mathur P, Singh V P, Dube B, Bajpai H S. Hypercoagulable state in diguglielmo's syndrome. J Postgrad Med 1976;22:157-9
| :: Introduction|| |
diGuglielmo's syndrome is a rare haematological disorder, which has aroused a lot of curiosity and controversy amongst the haematologists. ,, Studies on blood coagulation and fibrinolytic system which have been commented upon in other myeloproliferative disorders ,,, have not been well documented in diGuglielmo's syndrome.
In this paper we report a case of diGuglielmo's syndrome which was found to have hypercoagulable state.
| :: Case report|| |
A 28 year old Hindu house wife (S.D.) presented with intermittent episodes of fever (102-104°F), associated with weakness, anorexia, malaise, prostration, pain in abdomen and bleeding from gums for 2 months. On examination there was pallor, splenomegaly and hepatomegaly. A few cervical lymph nodes were palpable which were small, firm, discrete and non-tender. There was no bony tenderness.
The peripheral blood examination showed haemoglobin to be 7.5 gm%, total nucleated cell count 30,000,/Cu. mm. with mature neutrophils 26% , myeloblast 5%, metamyelocytes and myelocytes together 9%, lymphocytes 9%, eosinophils 1%, and erythroblasts 50 %, erythroid series of cells showed megaloblastoid character and unusually lobulated nuclei. The cells were of all stages including the basopholic stage. The platelet count was 20,000%Cu. mm. The red blood cells were mostly normocytic normochromic with mild macroovalocytosis. Reticulocyte count was 5.2%. Serum Vitamin B 12 and serum folates were within the normal range*. Liver function test revealed negative, Vandenbergh test; serum bilirubin was less than 0.5 mg %, thymol turbidity 4 units and alkaline phosphatase 12 K.A. units.
On admission, the coagulation tests revealed the following results, Kaolin cephalin clotting time was 36.0 sec. (control: 44 sec.), whole blood clotting time was 4 min 45 sec., prothrombin time 19.0 sec. (control: 18.5 sec.), thrombin time 10.0 sec. (control: 10.0 sec.) and bleeding time 7 min 30 sec. (normal 3-7 min.).
Tests for fibrinolysis: Euglobulin lysistime  was more than 300 minutes (normal 220 ± 40 min). Active plasmin and plasminogen activators were measured on fibrin plate.  active plasmin was not demonstrable while euglobulin fraction zone of lysis was 56 mm 2 (normal 238 ± 96 mm 2 ). Plasma plasminogen and available plasmin levels were estimated over heated fibrin plate  and results expressed as percentage of control. The reading were 125% and 106% respectively. Plasma fibrinogen  was 576 mg % (normal 294 ± 44 mg%) and serum F.D.P. levels  were 20 µg/ml (normal 2.25 ± 2.0 µg/ ml). Streptokinase inhibitors  were 125% of normal control.
The patient was put on 6-mercaptopurine (100 mg/day) and prednisolone (40 mg/day) therapy. After about a month, the peripheral blood showed haemoglobin to be 9.0 gm%, total leucocyte count 7,500,/ Cu. mm. and frank picture of acute myeloblastic leukemia with 56% of cells being myeloblasts and only 5% erythroblasts. At this stage, the patient was discharged on her request. She did not turnup for further follow-up.
| :: Discussion|| |
diGuglielmo's syndrome may be defined as a self perpetuating myeloproliferative disorder of undetermined origin characterised by progressive anaemia, striking erythroblastic hyperplasia of bone marrow of megaloblastic, megaloblastoid or normoblastic type, and the gradual development of increasing number of myaloblasts. Eventually in some cases, a fair number of myeloblasts are sufficient to warrant the diagnosis of erythroleukemia and later of myeloblastic leukemia.  It was also suggested to consider diGuglielmo's syndrome as a highly variable generalised myeloproliferative disorder in which erythremic myelosis, erythroleukemia and myeloblastic leukemia may all appear either sequentially or as a `mixed' form.  Scott et al.  described three patients, who were found to have a chronic form of myeloproliferative syndrome, prior to the development of erythroleukaemia. Two of these had chronic granulocytic leukaemia and the other had polycythemia vera. In the remaining cases the disease appeared to have an acute onset and the initial diagnosis was erythroleukaemia. Cur patient presented with mixed myeloid and erythroid proliferation but soon there was transition to frank leukaemic picture. The megaloblastoid feature was also quite prominent.
There was significant shortening of kaolin cephalin clotting time together with reduced plasminogen activators and elevated plasma fibrinogen levels. Other parameters of fibrinolysis were not much altered. These features are quite suggestive of a 'hypercoagulable state.  The insipient state of 'defibrination' was pointed out by the elevated levels of serum fibrinogen /fibrin degradation products, as described by Ogston et al. 
| :: Acknowledgement|| |
We are thankful to Prof. Dr. K. N. Udupa, Director Institute of Medical Sciences, for his kind permission to publish this case report.
| :: References|| |
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