INTRAVASCULAR OCCLUSION BY LEUKEMIC BLAST CELLS CAUSING MULTIPLEX HAND
POSTER 33
NECROSIS IN A PATIENT WITH ACUTE MYELOID LEUKEMIA
László Imre Pinczés1,2, Ferenc Magyari1,2, Gyula Reményi1, György Pfliegler3, Sándor Barna4, Judit
Bedekovics5 and Árpád Illés1,2
(1)Division of Hematology, Department of Internal Medicine, Faculty of Medicine, University of
Debrecen, Debrecen, Hungary
(2)Doctoral School of Clinical Medicine, University of Debrecen, Debrecen, Hungary
(3)Division of Rare Diseases, Department of Internal Medicine, Faculty of Medicine, University of
Debrecen, Debrecen, Hungary
(4)Scanomed Ltd., Debrecen, Hungary
(5)Department of Pathology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
It is well known that acute myeloid leukemia (AML) can be accompanied by embolic events, skin
lesions, and hyperviscosity. These complications, however, are the most frequent results of
thromboembolisation, extramedullary disease manifestation, and hyperleukocytosis, respectively.
We present the case of an AML patient with a sudden onset of skin lesions, where an unusual
etiology was confirmed.
Our patient had a history of hypertension and renal artery stenosis. In April 2017, at the age of 76,
she was diagnosed with AML (FAB M4). The disease had poor prognostic markers and complex
karyotype abnormalities. She had a chemorefractory disease after the first cycle of 7+3 induction
therapy. After receiving dismal results, she withdrew consent for further treatment. In May 2018,
azacitidine was initiated to reduce increasing pancytopenia and peripheral blast count. She was
admitted to the Department of Hematology two weeks after receiving the first cycle of azacitidine
with sudden-onset pain and bruising in the right middle finger and left thenar eminence. Acute
arterial and venous obstruction, bacterial- or thromboembolism, coagulation defect, and
autoimmune disease were all excluded by a detailed examination. Differential diagnosis included
extramedullary leukemia, vasculitis, Achenbach syndrome, and direct occlusion of small vessels by
circulating blast cells. Despite the best supportive care available, our patient experienced rapid
progression to skin necrosis and underwent surgical necrectomy. Histological examination of the
occluding material proved that blast cell aggregation led to microvascular thrombosis of the palmar
arteriovenous system. Technetium-99m hand perfusion scintigraphy confirmed the disturbance of
microcirculation in both hands. It is notable that white blood cell count was within the normal range
at the initial presentation of skin lesions. After surgical necrectomy, cytoreductive treatment
comprising of the combination of hydroxyurea and azacitidine decreased peripheral blast count and
led to a complete regression of skin symptoms.
Microcirculation disruption caused by circulating blast cells is a rarity in the literature of AML. With a
proposed median time to best response over a hundred days during azacitidine treatment, we
recommend further attempts at cytoreduction in patients with circulating blast cells – even in the
absence of hyperleukocytosis.