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Chronic Neutrophilic Leukemia


Presentation

  • Taken together with the observation that leukocytosis and hepatosplenomegaly in the present case subsided without specific treatment, we speculate that myeloid proliferation in the present case may have been a leukemoid reaction to underlying monoclonal[ncbi.nlm.nih.gov]
  • At initiation of therapy, both patients had progressive disease and presented with large tumors.[ncbi.nlm.nih.gov]
  • Gene rearrangements of the immunoglobulin heavy and kappa light chains were also present. To our knowledge, this is the first reported case of CNL associated with CLL.[ncbi.nlm.nih.gov]
  • Among the 30 cases reported so far, plus the four presented here, combined myelodysplastic features were observed in five cases and transformation into acute myelogenous leukemia in six.[ncbi.nlm.nih.gov]
  • A 60-year-old woman who presented with weakness, night sweats, bone pain, easy bruising and weight loss was found to have ecchymoses and hepatosplenomegaly.[ncbi.nlm.nih.gov]
Massive Splenomegaly
  • After anti-tuberculous therapy for 1 year, the patient underwent splenectomy for massive splenomegaly and progressive leukocytosis. The presence of the homozygous JAK2 V617F tyrosine kinase mutation was also demonstrated in the peripheral blood.[ncbi.nlm.nih.gov]
Gangrene
  • The clinical manifestations were characterized by hyperleukocyte syndrome with respiratory distress and ischemic legs with gangrene.[ncbi.nlm.nih.gov]
  • Gangrene of the leg progressed and amputation of the right leg, leaving the upper one third, was performed in January 1997.[jjco.oxfordjournals.org]
Intraabdominal Hemorrhage
  • Her postpartum course was complicated by intraabdominal hemorrhage and severe preeclampsia. She recovered with intensive medical and surgical management.[ncbi.nlm.nih.gov]
Suggestibility
  • Review of the literature suggests that such mosaicism is common in CNL, possibly accounting for the favorable prognosis observed in many patients with this disorder.[ncbi.nlm.nih.gov]
  • These results suggest that deficiency of CNL neutrophil function might be due to insufficiency of some inflammatory cytokine-specific signaling.[ncbi.nlm.nih.gov]
  • Our data suggest mutation analysis of CSF3R, SETBP1 and CALR should be included in the diagnostic criteria for CNL. These data may also have therapy implications.[ncbi.nlm.nih.gov]
  • A high neutrophil alkaline phosphatase score and a normal bone marrow cell karyotype suggested that the patient had chronic neutrophilic leukemia rather than chronic myeloid leukemia.[ncbi.nlm.nih.gov]
  • The patient showed sustained mature neutrophilic leukocytosis, splenomegaly, a high leukocyte alkaline phosphatase score, elevated serum vitamin B12 and uric acid, myeloid hyperplasia and absence of ph' chromosome in the bone marrow, with no evidence suggesting[ncbi.nlm.nih.gov]

Workup

  • The workup at that facility revealed a non-ST elevation myocardial infarction as well as profound leukocytosis (reported at 260x10 9 /L).[omicsonline.org]
  • The diagnosis of CML is based on the histopathologic findings in the peripheral blood and the Philadelphia chromosome in bone marrow cells (see Workup ). CML accounts for 20% of all leukemias affecting adults.[emedicine.medscape.com]
Bone Marrow with Hyperplasia
  • Chronic neutrophilic leukemia (CNL) is recognized as a distinct clinicopathologic entity characterized by sustained mature neutrophilic leukocytosis splenomegaly with bone marrow granulocytic hyperplasia without evidence of dysplasia or striking reticulin[ncbi.nlm.nih.gov]
  • Chronic neutrophilic leukemia (CNL) is a myeloproliferative neoplasm characterized by sustained, mature neutrophilic leukocytosis, splenomegaly, and bone marrow granulocytic hyperplasia.[ncbi.nlm.nih.gov]

Treatment

  • To our knowledge, this is the first case with CNL who showed complete response with cytogenic remission after treatment of imatinib.[ncbi.nlm.nih.gov]
  • The prognosis is generally poor and there is no consensus therapeutic strategy for the treatment of this disease.[ncbi.nlm.nih.gov]
  • The eruption progressed, despite treatment with topical and systemic corticosteroids.[ncbi.nlm.nih.gov]
  • Treatment with hydroxyurea and cytosine arabisonide was ineffective.[ncbi.nlm.nih.gov]
  • Herein, we discuss the potential impact of these findings for the classification and treatment of these disorders.[ncbi.nlm.nih.gov]

Prognosis

  • Current evidence shows that SETBP1 mutation is associated with a poor prognosis in patients with MDS and CMML, but not in patients with CNL.[ncbi.nlm.nih.gov]
  • Review of the literature suggests that such mosaicism is common in CNL, possibly accounting for the favorable prognosis observed in many patients with this disorder.[ncbi.nlm.nih.gov]
  • It remains to be established what role this mutation, which gives cells a proliferative advantage, might play in the pathogenesis and prognosis of rare atypical MPD.[ncbi.nlm.nih.gov]
  • Background literature and the prognosis of CNL are discussed.[ncbi.nlm.nih.gov]
  • Although the prognosis of CNL was not determined, curative therapy including allogeneic hematopoietic stem cell transplantation should be attempted in young patients with CNL.[ncbi.nlm.nih.gov]

Etiology

  • Etiology 90% of all cases show a normal genetic prophile, BCR-ABL1 is absent, however JAK 2 mutation may occasionally occure.[atlases.muni.cz]
  • View Article : Google Scholar : PubMed/NCBI 8 Sakka V, Tsiodras S, Giamarellos-Bourboulis EJ and Giamarellou H: An update on the etiology and diagnostic evaluation of a leukemoid reaction. Eur J Intern Med. 17:394–398. 2006.[spandidos-publications.com]

Epidemiology

  • T6181 Epidemiology [ edit ] This is a rare disease, with less than 100 cases reported.[en.wikipedia.org]
  • ., M.D. [1] Associate Editor(s)-in-Chief: Overview Historical Perspective Classification Pathophysiology Causes Differentiating Chronic neutrophilic leukemia from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications[wikidoc.org]
  • D47.1 Chronic myeloproliferative disease Corresponding ICD-10-CM Codes (U.S. only) D47.1 Chronic myeloproliferative disease (effective October 01, 2015) Signs and Symptoms Bleeding from mucocutaneous surfaces GI bleeding Progression and Transformation Epidemiology[seer.cancer.gov]
  • Epidemiology The American Cancer Society (ACS) estimates that 8430 new cases of CML will be diagnosed in 2018, 4980 in males and 3450 in females. The ACS estimates that 1090 deaths from CML will occur in 2018, 620 in males and 470 in females.[emedicine.medscape.com]
Sex distribution
Age distribution

Pathophysiology

  • Further studies including the molecular cloning of the t(15;19)(q13;p13.3) will be important in understanding the pathophysiology of CNL with a heterogeneous clinical course and the exploitation of the basic mechanisms of imatinib treatment.[ncbi.nlm.nih.gov]
  • Corresponding Author: [email protected] ACADEMIC EDITOR: Athavale Nandkishor, Associate Editor FUNDING: This research is conducted as part of QNRF sponsored project (Novel approach in Myeloproliferative Neoplasms what determines the Pathophysiology[insights.sagepub.com]
  • ., M.D. [1] Associate Editor(s)-in-Chief: Overview Historical Perspective Classification Pathophysiology Causes Differentiating Chronic neutrophilic leukemia from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications[wikidoc.org]
  • Neutrophil functions in CNL cases have been reported to vary, and they may be important in maintaining the function of the patient's immune system and in understanding the pathophysiology of CNL associated with MM.[synapse.koreamed.org]
  • Myeloproliferative Syndromes: Clinical: Addressing Areas of Unmet Need in Prognostic Assessments and Therapy for MPNs Source Type: research Interactions with the bone marrow microenvironment (BMM) influence the pathophysiology of hematological malignancies[medworm.com]

Prevention

  • Treatment and Prevention Until the first decade of this century, the treatment of CNL focused on disease control rather than cure.[diagnose-me.com]
  • Get optimal test results with guidance on error detection, correction, and prevention as well as cost-effective test selection.[books.google.com]
  • […] retinal diseases with sustained release corticosteroids US6239113B1 ( en ) 1999-03-31 2001-05-29 Insite Vision, Incorporated Topical treatment or prevention of ocular infections WO2000063168A1 ( en ) 1999-04-16 2000-10-26 Coelacanth Chemical Corporation[google.co.in]
  • Some people are fine for several years with only a daily aspirin to prevent blood clots and regular doctor visits.[webmd.com]
  • Thalidomide, lenalidomide, and pomalidomide are drugs that prevent blood vessels from growing into areas of tumor cells. See Drugs Approved for Myeloproliferative Neoplasms for more information.[northshore.org]

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