ISSN: 1300-7777 E-ISSN: 1308-5263
FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia: A Low-Burden Disease with Dramatic Response to Imatinib - A Report of 5 Cases from South India [Turk J Hematol]
Turk J Hematol. 2014; 31(1): 56-60 | DOI: 10.4274/Tjh.2013.0086  

FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia: A Low-Burden Disease with Dramatic Response to Imatinib - A Report of 5 Cases from South India

Anıl Kumar N.1, Vishwanath Sathyanarayanan1, Visweswariah Lakshmi Devi2, Namratha N. Rajkumar2, Umesh Das1, Sarjana Dutt3, Lakshmaiah K Chinnagiriyappa1
1Kidwai Memorial Institute Of Oncology, Department Of Medical Oncology, Karnataka, India
2Kidwai Memorial Institute Of Oncology, Department Of Pathology, Karnataka, India
3Oncquest Laboratories Ltd., New Delhi, India

OBJECTIVE: Eosinophilia associated with FIP1L1-PDGFRA rearrangement represents a subset of chronic eosinophilic leukemia and affected patients are sensitive to imatinib treatment. This study was undertaken to learn the prevalence and associated clinicopathologic and genetic features of FIP1L1-PDGFRA rearrangement in a cohort of 26 adult patients presenting with profound eosinophilia (>1.5x109/L).
METHODS: Reverse-transcriptase polymerase chain reaction and gel electrophoresis were used for the detection of FIP1L1-PDGFRA rearrangement.
RESULTS: Five male patients with splenomegaly carried the FIP1L1-PDGFRA gene rearrangement. All patients achieved complete hematological response within 4 weeks of starting imatinib. One patient had previous deep vein thrombosis and 1 patient had cardiomyopathy, which improved with steroids and imatinib. Conventional cytogenetics was normal in all these patients. No primary resistance to imatinib was noted.
CONCLUSION: This study indicates the need to do the FIP1L1-PDGFRA assay in patients with hypereosinophilic syndrome. Prompt treatment of this condition with imatinib can lead to complete hematological response and resolution of the organ damage that can be seen in this setting.

Keywords: PDGFRA, Chronic eosinophilic Leukemia, Imatinib, India


FIP1L1-PDGFRA Pozitif Kronik Eozinofilik Lösemi: İmatinib için Dramatik Yanıt ile Bir Düşük Yük Hastalığı-Güney Hindistan’dan 5 Olgu Sunumu

Anıl Kumar N.1, Vishwanath Sathyanarayanan1, Visweswariah Lakshmi Devi2, Namratha N. Rajkumar2, Umesh Das1, Sarjana Dutt3, Lakshmaiah K Chinnagiriyappa1
1Kidwai Memorial Institute Of Oncology, Department Of Medical Oncology, Karnataka, India
2Kidwai Memorial Institute Of Oncology, Department Of Pathology, Karnataka, India
3Oncquest Laboratories Ltd., New Delhi, India

AMAÇ: FIP1L1-PDGFRA rearranjmanı ilişkili eozinofili, kronik eozinofilik löseminin bir alt grubunu temsil eder ve etkilenen hastalar imatinib tedavisine duyarlıdır. Bu çalışma, şiddetli eozinofili ile başvuran 26 erişkin hastadan oluşan bir grupta FIP1L1-PDGFRA rearranjmanının klinikopatolojik ve genetik yaygınlığını, ve özelliklerini öğrenmek için yapıldı.
YÖNTEMLER: FIP1L1-PDGFRA rearranjmanının tespiti için revers-transkriptaz polimeraz zincir reaksiyonu ve jel elektroforezi kullanıldı.
BULGULAR: Splenomegaliye sahip beş erkek hasta FIP1L1-PDGFRA gen yeniden düzenlenmesini taşıyordu. İmatinib başlangıcından 4 hafta içinde tüm hastalarda tam hematolojik yanıt elde etti. Bir hastada önceden var olan derin ven trombozu ve 1 hastada steroid ve imatinib ile düzelen kardiyomiyopati vardı. Tüm bu hastalarda konvansiyonel sitogenetik normaldi. İmatinibe karşı birincil direnç kaydedilmedi.
SONUÇ: Bu çalışma hipereozinofilik sendromlu hastalarda FIP1L1-PDGFRA testinin yapılması gerektiğini gösterir. Imatinib ile bu durumun acil tedavisi tam hematolojik yanıta ve bu durumda görülebilecek organ hasarının engellenmesine yol açabilir.

Anahtar Kelimeler: PDGFRA, Kronik eozinofilik lösemi, İmatinib, Hindistan


Anıl Kumar N., Vishwanath Sathyanarayanan, Visweswariah Lakshmi Devi, Namratha N. Rajkumar, Umesh Das, Sarjana Dutt, Lakshmaiah K Chinnagiriyappa. FIP1L1-PDGFRA-Positive Chronic Eosinophilic Leukemia: A Low-Burden Disease with Dramatic Response to Imatinib - A Report of 5 Cases from South India. Turk J Hematol. 2014; 31(1): 56-60

Corresponding Author: Anıl Kumar N., India


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