A case of paraneoplastic hyperleukocytosis closely mimicking chronic neutrophilic leukemia

Mittal, Navkirti; Rahman, Khaliqur; Singh, Prashant; Panda, Ipsita; Nityanand, Soniya

doi:10.4103/0973-1482.199434

Cited by 2 publications

(3 citation statements)

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“…Most patients may go through an asymptomatic or minimally symptomatic period, characterized only by neutrophilic leukocytosis, with or without mild splenomegaly, lasting for several months or even years, before evolving to symptomatic disease. In many cases the disease may be discovered incidentally as unexplained persistent neutrophilia, after excluding any other potential causes as infections or inflammatory and neoplastic disorders, which occasionally can induce extreme hyperleukocytosis ( 22 , 23 ). Less commonly, patients may complain for fatigue and restlessness, weight loss, night sweats, pruritus, and mild bone pain or may exhibit various hemorrhagic manifestations and recurrent episodes of gout ( 24 ).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Chronic Neutrophilic Leukemia: A Comprehensive Review of Clinical Characteristics, Genetic Landscape and Management

et al. 2022

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Chronic neutrophilic leukemia (CNL) represents a rare disease, that has been classified among the BCR/ABL-negative myeloproliferative neoplasms. The disease is characterized by marked leukocytosis with absolute neutrophilia and its clinical presentation may vary from asymptomatic to highly symptomatic with massive splenomegaly and constitutional symptoms. CNL prognosis remains relatively poor, as most patients succumb to disease complications or transform to acute myeloid leukemia. Recent studies have demonstrated that CSF3R mutations drive the disease, albeit the presence of other secondary mutations perplex the genetic landscape of the disease. Notably, the presence of CSF3R mutations has been adopted as a criterion for diagnosis of CNL. Despite the vigorous research, the management of the disease remains suboptimal. Allogeneic stem cell transplantation represents the only treatment that could lead to cure; however, it is accompanied by high rates of treatment-related mortality. Recently, ruxolitinib has shown significant responses in patients with CNL; however, emergence of resistance might perturbate long-term management of the disease. The aim of this review is to summarize the clinical course and laboratory findings of CNL, highlight its pathogenesis and complex genetic landscape, and provide the context for the appropriate management of patients with CNL.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Chronic Neutrophilic Leukemia: A Comprehensive Review of Clinical Characteristics, Genetic Landscape and Management

et al. 2022

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“…The most commonly reported symptoms are fatigue and hepatosplenomegaly, while the development of lymphadenopathy has remained uncommon in the presentation of CNL [4,5]. Exclusion of other causes of neutrophilia is required for the diagnosis of CNL [16]. Reactive neutrophilia may be present in infectious, inflammatory, and neoplastic conditions [8].…”

Section: Disease Features and Diagnosismentioning

confidence: 99%

“…Reactive neutrophilia may be present in infectious, inflammatory, and neoplastic conditions [8]. Plasma cell neoplasms may also present with secondary neutrophilia, whereas solid malignancies of the lung, connective tissues, and urogenital tract can lead to the development of paraneoplastic hyperleukocytosis [8,16]. Mature neutrophils are also a feature of other myeloid malignancies such as atypical CML (aCML) and chronic myelomonocytic leukemia (CMML) [8].…”

Section: Disease Features and Diagnosismentioning

confidence: 99%

Chronic Neutrophilic Leukemia: A Literature Review of the Rare Myeloproliferative Pathology

Anil

Gosal

Kaur

et al. 2021

Cureus

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Hematological malignancies often develop due to a vast spectrum of environmental and genetic etiologies. Chronic neutrophilic leukemia (CNL) can be described as a BCR-ABL1 (Philadelphia chromosome)-negative myeloproliferative neoplastic disease with various genetic mutations that may directly or indirectly play a role in its pathogenesis. A well-established mutation in CNL is the CSF3R (a cytokine receptor) which has been incorporated into the diagnostic criteria for the disease. However, evidence of other mutations such as SETBP1, ASXL1, and TET2 has also shed more light on the pathogenesis of this condition. Due to the unknown incidence and heterogeneous presentation of the disease, the diagnosis and management are often difficult and lack satisfactory data. The purpose of this review is to yield further insight into a disease that lacks awareness in the medical community. Using PubMed as a database, relevant studies and case reports were reviewed. The data compiled were used to acknowledge the disease in terms of etiology, clinical manifestation, molecular pathogenesis, and available treatment modalities. Though existing treatment modalities have been shown to induce clinical improvement, the outcomes are not reliable, and further research is required to reach a comprehensive "standard of care" for the disease.

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A case of paraneoplastic hyperleukocytosis closely mimicking chronic neutrophilic leukemia

Cited by 2 publications

References 0 publications

Chronic Neutrophilic Leukemia: A Comprehensive Review of Clinical Characteristics, Genetic Landscape and Management

Chronic Neutrophilic Leukemia: A Comprehensive Review of Clinical Characteristics, Genetic Landscape and Management

Chronic Neutrophilic Leukemia: A Literature Review of the Rare Myeloproliferative Pathology

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