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  IN THIS Article
 ::  Abstract
 ::  Introduction
 ::  Case report
 ::  Discussion
 ::  Acknowledgement
 ::  References

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Year : 1976  |  Volume : 22  |  Issue : 3  |  Page : 157-159

Hypercoagulable state in diguglielmo's syndrome

Institute of Medical Sciences, Banaras Hindu University, Varanasi-5, India

Correspondence Address:
Pradeep Mathur
Institute of Medical Sciences, Banaras Hindu University, Varanasi-5
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Source of Support: None, Conflict of Interest: None

PMID: 1076379

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 :: Abstract 

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

How to cite this URL:
Mathur P, Singh V P, Dube B, Bajpai H S. Hypercoagulable state in diguglielmo's syndrome. J Postgrad Med [serial online] 1976 [cited 2023 May 31];22:157-9. Available from:

 :: Introduction Top

diGuglielmo's syndrome is a rare hae­matological disorder, which has aroused a lot of curiosity and controversy amongst the haematologists. [4],[12],[13] Studies on blood coagulation and fibrinolytic system which have been commented upon in other myeloproliferative disorders [5],[7],[9],[11] 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 Top

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 [2] was more than 300 minutes (normal 220 ± 40 min). Active plasmin and plas­minogen activators were measured on fibrin plate. [1] 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 [3] and results expressed as percentage of control. The reading were 125% and 106% respectively. Plasma fibrinogen [10] was 576 mg % (normal 294 ± 44 mg%) and serum F.D.P. levels [6] were 20 µg/ml (normal 2.25 ± 2.0 µg/ ml). Streptokinase inhibitors [8] 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 Top

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. [4] 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. [4] Scott et al. [12] 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. [14] The insipient state of 'defibrination' was point­ed out by the elevated levels of serum fibrinogen /fibrin degradation products, as described by Ogston et al. [7]

 :: Acknowledgement Top

We are thankful to Prof. Dr. K. N. Udupa, Director Institute of Medical Sciences, for his kind permission to pub­lish this case report.

 :: References Top

1.Astrup, T. and Mullertz, S.: The fibrin­plate method for estimating fibrinolytic activity. Arch. Biochem., 40: 346-351,1952.  Back to cited text no. 1    
2.Biggs, R. and Macfarlane, R. G.: Blood coagulation and its disorders. Blackwell Scientific Publication, Oxford and Edin­ burgh, p. 395, 1962.  Back to cited text no. 2    
3.Bishop, R„ Ekert, N., Gilchrist, G., Shan-born, E. and Fekete. L.: The preparation and evaluation of a standardized fibrin plate for assessment of fibrinolytic acti­vity. Thromb. Diath. Haemorrh, 23: 202­210, 1970.  Back to cited text no. 3    
4.Demeshek, W.: The diGuglielmo's syn­drome revisited. Blood, 34: 567-572, 1969.  Back to cited text no. 4    
5.Gralnick, H. R., Bagley. J. and Abrell, E.: Heparin treatment for the haemor­rhagic diathesis of acute promyelocytic leukaemia. Amer. J. Med., 52: 167, 1971.  Back to cited text no. 5    
6.Merskey, C., Lalesari, P. and Johnson, A.: A rapid, simple, sensitive method for measuring fibrinolytic split products of human serum. Proc. Soc. Exp. Biol. Med., 131: 871-875, 1969.  Back to cited text no. 6    
7.Ogston, D., Dawson, A. A. and Adam. H. M.: The Fibrinolytic enzyme system in leukaemia, myelomatosis and myelo proliferattve diseases. Acta. Haematol.. 48: 322-330, 1972.  Back to cited text no. 7    
8.Paraskevasa. M., Nileson. I. M. and Mar­tinson, G.: A method for determining serum inhibitors of plasminogen activa­tion. Scand. J. Clin. Lab. Invest., 14: 138-144, 1962.  Back to cited text no. 8    
9.Pochedly, C., Miller, S. P. and Mehta, A.: Hypercoagulable state in Children with acute leukaemia or dissiminated solid tumors. Oncology, 28: 517, 1973.  Back to cited text no. 9    
10.Quick, A. G.: Haemorrhagic diseases and thrombosis. Lee & Febriger (Philadel­phia), p. 410, 1966.  Back to cited text no. 10    
11.Rosenthal, R. L.: Acute promyelocytic leukaemia associated with hypofibrino­genemia. Blood, 21: 495-508, 1963.  Back to cited text no. 11    
12.Scott, R. B., Ellison, R. R. and Ley, A. B.: A Clinical Study of twenty cases of erythroleukaemia (diGuglemo's syn­drome), Amer. J. Med., 37: 162-171, 1964.  Back to cited text no. 12    
13.Sheets, R. F., Drevets, C. C. and Hamil­ton, H. E.; Erythroleukaemia (diGugli­elme Syndrome). Arch. Int. Med., III: 295- 306, 1963.  Back to cited text no. 13    
14.Sise, H. S., Moschos, C. B. and Becker, R.: On the nature of Hypercoagulability. American Journal of Medicine, 33: 667-678, 1962.  Back to cited text no. 14    


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