[Atypical defibrination syndromes and acute leukemias with a t(9,22) translocation, apropos of 2 cases].
We report two cases of atypical defibrination syndromes in patients with respectively acute monoblastic leukemia (chronic myeloid leukemia initially) and acute lymphoblastic leukemia. Hemostasis studies show low fibrinogen level, elevated D-dimers, decreased alpha 2 antiplasmin and factor V, normal antithrombin III values. Plasminogen is below the normal range in one patient. Soluble complexes, which are an important argument for diagnosis of intravascular coagulation disease, are not detected in both patients. Primary or secondary hyperfibrinolysis seems also excluded since euglobulin clot lysis time was normal. Enzymatic proteolysis of fibrinogen (or fibrin) by the blast cells has been reported by some authors; this mechanism could account for the hemostasis abnormalities observed in these two patients.
Meddeb
B
B
Service d'hématologie, hôpital Aziza Othmana, place du Gouvernement, La Kasbah, 1007 Tunis, Tunisie.
Guermazi
S
S
Hafsia
R
R
Ben Abid
H
H
Gouider
E
E
Ben Lakhal
R
R
Bel Haj Ali
Z
Z
Ben Othman
T
T
Jeddi
R
R
Dellagi
K
K
Hafsia
A
A
D000328:Adult; D000990:Antithrombin III / Q000032:analysis; D003937:Diagnosis, Differential; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis; D005166:Factor V Deficiency / Q000097:blood; D005337:Fibrin / Q000172:deficiency*; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D005340:Fibrinogen / Q000032:analysis* / Q000378:metabolism; D005342:Fibrinolysis; D006801:Humans; D007948:Leukemia, Monocytic, Acute / Q000097:blood*; D008297:Male; D014411:Neoplastic Stem Cells / Q000378:metabolism; D010958:Plasminogen / Q000032:analysis; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000097:blood*; D013577:Syndrome; D000979:alpha-2-Antiplasmin / Q000172:deficiency
D005338:Fibrin Fibrinogen Degradation Products; D000979:alpha-2-Antiplasmin; C036309:fibrin fragment D; D000990:Antithrombin III; D005337:Fibrin; D005340:Fibrinogen; D010958:Plasminogen