Loeffler endocarditis in chronic eosinophilic leukemia with FIP1L1/PDGFRA rearrangement: full recovery with low dose imatinib

Kim, Dae Sik; Lee, Sunki; Choi, Chul Won

doi:10.3904/kjim.2016.100

Cited by 4 publications

(3 citation statements)

References 5 publications

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“…Our patient is a case of chronic eosinophilic leukemia associated with PDGFRA rearrangement, presenting with severe eosinophilia and evidence of multi-organ damage. Several case reports and case series of patients with eosinophilia associated with FIP1L1/PDGFRA rearrangement, have been published from various countries [7][8][9][10][11][12][13][14][15][16][17][18]. In addition to the more common presentations, they have described the clinical features and outcome of this rare disease entity in paediatric patients, the occurrence of rare FIP1L1 breakpoint (located outside the usual breakpoint region) and atypical presentation of the disease with thrombotic thrombocytopenia purpura.…”

Section: Discussionmentioning

confidence: 99%

Severe Eosinophilia Associated with FIP1L1/PDGFRA Rearrangement Presenting with Yamaguchi Syndrome

Aamir¹,

Meraj²,

Hm³

2021

annhematoloncol

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FIP1L1/PDGFRA is a rare genetic rearrangement, presenting most commonly as Chronic Eosinophilic Leukemia (CEL), but may also be associated with other myeloid and lymphoid neoplasms. The peripheral blood and bone marrow exhibit a striking eosinophilia, often associated with an increased number of mast cells on trephine biopsy. Tissue infiltration by eosinophils and release of cytokines from eosinophilic granules mediate multi-organ tissue damage. The tyrosine kinase inhibitor Imatinib has been shown to induce rapid and complete clinical and haematological responses in patients harboring the mutation. We present the case of a young patient with CEL associated with PDGFRA rearrangement, presenting with severe eosinophilia and evidence of multi-organ damage (cardiac, renal, endocrine and respiratory). The peripheral blood and bone marrow displayed a striking eosinophila, and FISH analysis for FIP1L1/PDGFRA revealed a positive fusion signal in 92% of the nuclei examined. Echocardiography showed left ventricular apical hypertrophy (Yamaguchi syndrome), which has previously not been reported in this subset of patients. He was managed with supportive care, along with low-dose imatinib (100mg/day initially), to which he achieved a rapid clinical and haematological response. Currently, five months from the initial diagnosis, he is doing well on low dose imatinib (100 mg) twice a week.

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Section: Discussionmentioning

confidence: 99%

Severe Eosinophilia Associated with FIP1L1/PDGFRA Rearrangement Presenting with Yamaguchi Syndrome

Aamir¹,

Meraj²,

Hm³

2021

annhematoloncol

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“…Für Patienten mit MLN-Eo und Nachweis des FIP1L1-PDGFRA-Fusionsgens ist ein sehr gutes Ansprechen mit schneller und anhaltender hämatologischer Remission auf den selektiven Tyrosinkinase-Inhibitor Imatinib gezeigt worden [4,5,[8][9][10][11].…”

Section: Diskussionunclassified

“…Im Gegensatz zur raschen hämatologischen Erholung sind begleitende kardiale Veränderungen jedoch teilweise nicht reversibel [12][13][14], auch wenn einige Fallberichte unter Imatinib eine hervorragende Regression der Löffler-Endokarditis-spezifischen Veränderungen im kurzfristigen Verlauf beschreiben [8,9,15]. So haben Kleinfeldt et al den Verlauf eines Hypereosinophilie-Syndroms mit kardialer Beteiligung unter Imatinib bereits mit 6 Monaten Nachbeobachtung beschrieben [16].…”

Section: Diskussionunclassified

Komplette Remission einer Löffler-Endokarditis bei einer myeloischen und lymphatischen Neoplasie mit Eosinophilie unter Imatinib

Wienemann

Bröckelmann

Adam

2020

Dtsch med Wochenschr

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Zusammenfassung Anamnese Ein 53-jähriger Patient stellte sich mit massivem Pruritus bei Ganzkörperexanthem, generalisierten Muskelschmerzen und Belastungsdyspnoe NYHA II vor. Untersuchungen und Diagnose Durch weiterführende hämatologische Untersuchungen wurde die Diagnose einer myeloischen und lymphatischen Neoplasie mit Eosinophilie (MLN-EO) und Nachweis eines FIPL1L1-PDGFRA-Fusionsgens gestellt. Im Rahmen der kardiologischen Diagnostik erfolgte eine echokardiografische Untersuchung mit Nachweis eines Thrombus im rechten Ventrikel. In der Magnetresonanztomografie (MRT) zeigte sich der intrakavitäre Thrombus vom Apex bis zum rechtsventrikulären Ausflusstrakt reichend. Therapie und Verlauf Eine orale Antikoagulation mit Phenprocoumon und eine zielgerichtete Therapie mit Imatinib wurden initiiert. Hierunter konnte in den 11- und 23-monatigen Verlaufskontrollen mittels Magnetresonanztomografie der Thrombus nachweislich aufgelöst werden. Der weitere Verlauf gestaltete sich komplikationslos. Folgerung Eine Löffler-Endokarditis mit begleitendem Ventrikelthrombus auf dem Boden einer myeloischen und lymphatischen Neoplasie mit Eosinophilie kann durch orale Antikoagulation und Therapie mit Imatinib erfolgreich behandelt werden.

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Loeffler endocarditis with intracardiac thrombus: case report and literature review

Zhang

et al. 2021

BMC Cardiovasc Disord

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Background Loeffler endocarditis is a relatively rare and potentially life-threatening heart disease. This study aimed to identify the characteristic features of Loeffler endocarditis with intracardiac thrombus on a background of hypereosinophilic syndrome (HES). Case presentation We described a 57-year-old woman with Loeffler endocarditis and intracardiac thrombus initially presenting with neurological symptoms, who had an embolic stroke in the setting of HES. After cardiac magnetic resonance (CMR), corticosteroids and warfarin were administered to control eosinophilia and thrombi, respectively. During a 10-month follow-up, the patient performed relatively well, with no adverse events. We also systematically searched PubMed and Embase for cases of Loeffler endocarditis with intracardiac thrombus published until July 2021. A total of 32 studies were eligible and included in our analysis. Further, 36.4% of recruited patients developed thromboembolic complications, and the mortality rate was relatively high (27.3%). CMR was a powerful noninvasive modality in providing diagnostic and follow-up information in these patients. Steroids were administered in 81.8% of patients, achieving a rapid decrease in the eosinophil count. Also, 69.7% of patients were treated with anticoagulant therapy, and the thrombus was completely resolved in 42.4% of patients. Heart failure and patients not treated with anticoagulation were associated with poor outcomes. Conclusions Cardiac involvement in HES, especially Loeffler endocarditis with intracardiac thrombus, carries a pessimistic prognosis and significant mortality. Early steroids and anticoagulation therapy may be beneficial once a working diagnosis is established. Further studies are needed to provide evidence-based evidence for managing this uncommon manifestation of HES.

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Loeffler endocarditis in chronic eosinophilic leukemia with FIP1L1/PDGFRA rearrangement: full recovery with low dose imatinib

Cited by 4 publications

References 5 publications

Severe Eosinophilia Associated with FIP1L1/PDGFRA Rearrangement Presenting with Yamaguchi Syndrome

Severe Eosinophilia Associated with FIP1L1/PDGFRA Rearrangement Presenting with Yamaguchi Syndrome

Komplette Remission einer Löffler-Endokarditis bei einer myeloischen und lymphatischen Neoplasie mit Eosinophilie unter Imatinib

Loeffler endocarditis with intracardiac thrombus: case report and literature review

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