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A UNIQUE PRESENTATION OF ACUTE PROMYELOCYTIC LEUKEMIA: AORTOILIAC OCCLUSIVE DISEASE (LERICHE SYNDROME)
Author(s): ,
Selin Berk
Affiliations:
Haematology,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
,
Murat Günaltılı
Affiliations:
Internal Medicine,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
,
Sevil Sadri
Affiliations:
Haematology,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
,
Ahmet Emre Eşkazan
Affiliations:
Haematology,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
,
Zafer Başlar
Affiliations:
Haematology,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
Teoman Soysal
Affiliations:
Haematology,Istanbul University Cerrahpasa Medical Faculty,Istanbul,Turkey
(Abstract release date: 05/18/17) EHA Library. Berk S. 05/18/17; 182415; PB1701
Selin Berk
Selin Berk
Contributions
Abstract

Abstract: PB1701

Type: Publication Only

Background
Acute promyelocytic leukemia (APL), FAB M3 subgroup of acute myeloid leukemia is known for its association with haemostatic disorders. Compared to bleeding thrombosis is a less commonly encountered complication of APL. Thrombosis of major arteries is a rare form of presentation.

Aims
A case, who applied with acute lower limb ischemia and diagnosed with APL and aortoiliac occlusive disease (Leriche syndrome), is presented.

Methods
A 53-year-old female patient presented with weakness, loss of appetite and pain in the lower extremities. She had diabetes mellitus (DM) regulated with metformin, hyperlipidemia (HL), and smoking history. Physical examination revealed general paleness and ischemia around big toe of the right foot. Laboratory studies revealed leukopenia, neutropenia, anemia, thrombocytopenia, elevated D-Dimer. A bone marrow aspiration and biopsy was done to enlighten the etiology of pancytopenia. The pathological examination of the bone marrow revealed abundant granular blasts (78%) and Auer rodes. The patient was diagnosed with APL, hypergranular classical form. t(15;17) was positive with fluorescence in situ hybridization. All-trans retinoic acid (ATRA) plus idarubicin treatment was started. In few days findings of ischemia progressed and encompassed 2nd, 4th and 5th toes together with the big toe (Figure on the left). Monophasic flow pattern (proximal stenosis?) was detected in bilateral common femoral arteries in lower extremity venous doppler ultrasonography. On CT angiography, abdominal aorta and bilateral common iliac arteries were observed to be occluded from L3 vertebra level till 1.5 cm after aortic bifurcation (Figure on the right). Low-molecular-weight heparin therapy was started. According to rheumatological tests and tests for lupus anticoagulant, anticardiolipin and antiphospholipid antibodies, anti-beta-2 glycoprotein-1, protein C-S, Antithrombin III and homocysteine levels, methylenetetrahydrofolate reductase, Factor V Leiden and prothrombin gene mutations no cause of tendency to thrombophilia could be determined. Echocardiography was normal. The patient was transferred to Cardiovascular Surgery Department for axillofemoral bypass operation.

Results
In APL 80% of thrombotic events occur before treatment or during induction. Acute lower limb ischemia as an initial feature of APL is very rare which makes our case unique. Thrombotic risk factors in APL include high leukocyte count, presence of coagulation disorder, ATRA+chemotherapy+antifibrinolytic therapy and ATRA syndrome. None of these were seen in the presented case. The effects of known predisposing risk factors to thrombosis meaning DM, HL and smoking cannot be ruled out. But development of acute thrombosis concomitant with APL diagnosis points out to the relationship between these two entities.

Conclusion
Current literature knowledge is based on case reports and 9 patients with APL who presented with acute lower limb ischemia were reported yet. As far as we know our case is the first APL case presenting with aortoiliac occlusive disease (Leriche syndrome).

Session topic: 4. Acute myeloid leukemia - Clinical

Keyword(s): Arterial Thrombosis, Acute Promyelocytic Leukemia

Abstract: PB1701

Type: Publication Only

Background
Acute promyelocytic leukemia (APL), FAB M3 subgroup of acute myeloid leukemia is known for its association with haemostatic disorders. Compared to bleeding thrombosis is a less commonly encountered complication of APL. Thrombosis of major arteries is a rare form of presentation.

Aims
A case, who applied with acute lower limb ischemia and diagnosed with APL and aortoiliac occlusive disease (Leriche syndrome), is presented.

Methods
A 53-year-old female patient presented with weakness, loss of appetite and pain in the lower extremities. She had diabetes mellitus (DM) regulated with metformin, hyperlipidemia (HL), and smoking history. Physical examination revealed general paleness and ischemia around big toe of the right foot. Laboratory studies revealed leukopenia, neutropenia, anemia, thrombocytopenia, elevated D-Dimer. A bone marrow aspiration and biopsy was done to enlighten the etiology of pancytopenia. The pathological examination of the bone marrow revealed abundant granular blasts (78%) and Auer rodes. The patient was diagnosed with APL, hypergranular classical form. t(15;17) was positive with fluorescence in situ hybridization. All-trans retinoic acid (ATRA) plus idarubicin treatment was started. In few days findings of ischemia progressed and encompassed 2nd, 4th and 5th toes together with the big toe (Figure on the left). Monophasic flow pattern (proximal stenosis?) was detected in bilateral common femoral arteries in lower extremity venous doppler ultrasonography. On CT angiography, abdominal aorta and bilateral common iliac arteries were observed to be occluded from L3 vertebra level till 1.5 cm after aortic bifurcation (Figure on the right). Low-molecular-weight heparin therapy was started. According to rheumatological tests and tests for lupus anticoagulant, anticardiolipin and antiphospholipid antibodies, anti-beta-2 glycoprotein-1, protein C-S, Antithrombin III and homocysteine levels, methylenetetrahydrofolate reductase, Factor V Leiden and prothrombin gene mutations no cause of tendency to thrombophilia could be determined. Echocardiography was normal. The patient was transferred to Cardiovascular Surgery Department for axillofemoral bypass operation.

Results
In APL 80% of thrombotic events occur before treatment or during induction. Acute lower limb ischemia as an initial feature of APL is very rare which makes our case unique. Thrombotic risk factors in APL include high leukocyte count, presence of coagulation disorder, ATRA+chemotherapy+antifibrinolytic therapy and ATRA syndrome. None of these were seen in the presented case. The effects of known predisposing risk factors to thrombosis meaning DM, HL and smoking cannot be ruled out. But development of acute thrombosis concomitant with APL diagnosis points out to the relationship between these two entities.

Conclusion
Current literature knowledge is based on case reports and 9 patients with APL who presented with acute lower limb ischemia were reported yet. As far as we know our case is the first APL case presenting with aortoiliac occlusive disease (Leriche syndrome).

Session topic: 4. Acute myeloid leukemia - Clinical

Keyword(s): Arterial Thrombosis, Acute Promyelocytic Leukemia

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