Presentation
Sixty-seven percent of patients presented with myeloid immunophenotype and 29% presented with a lymphoid immunophenotype. [healio.com]
Our cases indicate rare presentations of CML-BP with an extremely short chronic phase. Ph-positive de novo acute leukemia should be carefully distinguished from CML-BP. [ncbi.nlm.nih.gov]
Surprisingly, no extramedullary blast crises of B- lymphoid lineage have been reported before among cases of CML as the initial presentation. [mattioli1885journals.com]
Immune System
- Splenomegaly
In this study, we present a JAK2V617F positive MPN patient who experienced fatigue and splenomegaly, transforming into JAK2V617F negative AML. A diagnosis of acute monocytic leukemia was made in MPN blast phase. [ncbi.nlm.nih.gov]
Ultrasonography revealed splenomegaly. Laboratory tests showed white blood cell (WBC) count of 41.9×10 9 /L, Hb level of 11.2 g/dL, and platelet count of 74×10 9 /L. [synapse.koreamed.org]
Medical Definition of blast crisis : the terminal stage of chronic myelogenous leukemia that is characterized by a marked increase in the proportion of blast cells, by fever and pain in the bones, and by increased severity of anemia, thrombocytopenia, and splenomegaly [merriam-webster.com]
Blast phase of chronic myelogenous leukemia (CML), which can be discovered incidentally on CBC or in assessment of patients with symptoms and signs such as fever, fatigue, weight loss, anemia, thrombocytopenia, or splenomegaly. [online.epocrates.com]
- Massive Splenomegaly
The findings of massive splenomegaly, basophilia, and thrombocytosis point toward CML. Our patient had massive splenomegaly and basophilia but a normal platelet count. [ecancer.org]
He was found to have massive splenomegaly but no other physical findings. Laboratory showed a WBC of 140 × 10e 9 /L, Hb 68 gm/L, platelets of 97 × 10e 9 /L with a left shift granulopoiesis. His Sokal score was 1.1 (intermediate risk). [omicsonline.org]
Given the presence of constitutional symptoms and massive splenomegaly, the patient inquired about treatment with the JAK inhibitor ruxolitinib. [bloodjournal.org]
Hematological
- Easy Bruising
In October 2012, approximately four years after the initiation of therapy, the patient experienced a new onset of intermittent fever and easy bruising. [spandidos-publications.com]
Individuals with more advanced cases of CML may appear sickly and experience fevers, easy bruising, and bone pain. [healthcommunities.com]
Three months later in July 2011, the patient had new symptoms of easy bruising. Laboratory analysis showed a WBC 37.8, hemoglobin 10.9, and platelets 140,000. [managingcml.com]
Easy bruising. Fever. Gout due to rapid cell turnover. [patient.info]
Entire Body System
- Fever
Called also developmental crisis. myasthenic crisis the sudden development of dyspnea requiring respiratory support in myasthenia gravis ; the crisis is usually transient, lasting several days, and accompanied by fever. oculogyric crisis a symptom of [medical-dictionary.thefreedictionary.com]
In December 2015, he presented at our hospital with fever and lumbago. A complete blood count revealed a white blood cell count of 30,500/µL with 60% blasts, leading to diagnosis of SBP. [ncbi.nlm.nih.gov]
When tiredness, fever, and an enlarged spleen occur during the blastic phase, it is called blast crisis. [en.wikipedia.org]
- Anemia
Two patients achieved hematologic responses of 14 and 3 months and survived 19 and 6 months, respectively, while the non-responder died 2 months later. 2-CdA-induced anemia and thrombocytopenia, generally mild and reversible, were observed in all patients [ncbi.nlm.nih.gov]
[…] with a marked–30+% of WBCs—↑ proportion of blasts and number of lymphocytes or myelocytes in circulation and BM Clinical Lymphadenopathy, hepatosplenomegaly, spleen and bone pain, fever, thrombosis Lab Leukocytosis, thrombocytosis or thrombocytopenia, anemia [medical-dictionary.thefreedictionary.com]
Medical Definition of blast crisis : the terminal stage of chronic myelogenous leukemia that is characterized by a marked increase in the proportion of blast cells, by fever and pain in the bones, and by increased severity of anemia, thrombocytopenia, [merriam-webster.com]
- Weight Loss
Common features include excessive tiredness (fatigue), fever, and weight loss. Many affected individuals develop an enlarged spleen (splenomegaly), which can cause a feeling of fullness in the abdomen and a loss of appetite. [icdlist.com]
Blast phase of chronic myelogenous leukemia (CML), which can be discovered incidentally on CBC or in assessment of patients with symptoms and signs such as fever, fatigue, weight loss, anemia, thrombocytopenia, or splenomegaly. [online.epocrates.com]
Most patients will begin to feel fatigued and also experience weight loss, enlargement of the spleen, and pain in the trunk of the body. [news-medical.net]
These patients often have fever, poor appetite, and weight loss. In this phase, the CML acts a lot like an acute leukemia. [cancer.org]
- Fatigue
In this study, we present a JAK2V617F positive MPN patient who experienced fatigue and splenomegaly, transforming into JAK2V617F negative AML. A diagnosis of acute monocytic leukemia was made in MPN blast phase. [ncbi.nlm.nih.gov]
Blast phase of chronic myelogenous leukemia (CML), which can be discovered incidentally on CBC or in assessment of patients with symptoms and signs such as fever, fatigue, weight loss, anemia, thrombocytopenia, or splenomegaly. [online.epocrates.com]
In the accelerated phase, the number of CML cells is growing faster and causing symptoms such as fatigue, fever, weight loss and an enlarged spleen. If untreated, accelerated phase CML will eventually transform to blast phase CML. [lls.org]
Most patients will begin to feel fatigued and also experience weight loss, enlargement of the spleen, and pain in the trunk of the body. [news-medical.net]
If you have symptoms, they may include: Fatigue Weight loss Night sweats Fever Pain or a feeling of fullness below the ribs on the left side Tests that examine the blood and bone marrow diagnose CML. [icdlist.com]
- Pallor
On examination, pallor was present. CNS examination revealed E2M2V4; he was in altered sensorium, the power decreased on the left half of the body (2/5), and left plantar was up going. [ncbi.nlm.nih.gov]
On examination, the patient had pallor and splenic enlargement, measuring 22.9 cm in ultrasonography. Physical examination was otherwise unremarkable. [ecancer.org]
Gastrointestinal
- Abdominal Pain
It can be a result of encephalitis or a reaction to antipsychotic medications. tabetic crisis a painful paroxysm occurring in tabes dorsalis. vaso-occlusive crisis a sickle cell crisis in which there is severe pain due to infarctions in the bones, joints [medical-dictionary.thefreedictionary.com]
Some patients may also experience extreme fatigue, shortness of breath, and abdominal pain. Blast crisis closely resembles acute leukemia and has a survival range of three to six months. [nationalcmlsociety.org]
pain Bone pain Enlarged spleen Poor appetite and weight loss Bleeding Infections. [lls.org]
Skin
- Petechiae
It may also present with mild fever and night sweats due to an elevated basal level of metabolism. [4] Some ( petechiae and ecchymosis. [4] In these patients fevers are most commonly the result of opportunistic infections. [4] Some patients are initially [en.wikipedia.org]
Neurologic
- Headache
In June 2015, he started developing headaches that lasted for several hours. He had a normal brain CT scan and subsequently started to develop double vision, nausea, and ringing in his ears. [omicsonline.org]
Case Report A 55-year-old male patient presented to emergency department with a history of sudden weakness of left half of the body along with headache, altered sensorium, and deviation of angle of mouth to the right side since 1-day. [ncbi.nlm.nih.gov]
CASE 2: An 8-yr-old girl presented with headache and myalgia persisting for a week. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. [synapse.koreamed.org]
Workup
Diagnostic workup should include a complete blood cell count (CBC) with differential, bone marrow analysis with conventional cytogenetics, flow cytometry to determine whether the blast phase is of myeloid or lymphoid origin, and molecular mutational analysis [mdedge.com]
workup: (R) CD4, CD8, CD45; (S) CD5, CD3, CD45; (T) CD1a, CD2, CD45; (U) TdT, CD3, CD45; (V) CD3 (cyto), CD19, CD45 Miscellaneous: (W) CD4, CD14, CD45; (X) CD13, CD45; (Y) cIgG1, cIgG2a, CD45 CD45, CD2, CD3, and CD8 were used at 2.5 μL. [academic.oup.com]
Based on the patient’s presentation and lab results, further workup (CXR, Blood cultures, UA, etc) and treatment with appropriate fluid resuscitation and antibiotics should be considered and discussed with the oncology team. [emdocs.net]
Treatment
Treatment with CsA yielded no improvement in treatment outcome as measured by the frequency of induction resistance (68% vs 53%), rate of complete remission or restored chronic phase (CR/CP, 8% vs 30%), and survival (3 vs 5 months). [ncbi.nlm.nih.gov]
Prognosis
Imatinib resistance is an important hurdle in the treatment of chronic myeloid leukemia (CML), and CML patients with this drug resistance are often given a dismal prognosis. [ncbi.nlm.nih.gov]
Age—Patients 60 years of age or older have a less favorable prognosis. Spleen size—A patient with an enlarged spleen has a less favorable prognosis. [lls.org]
Etiology
Acute/etiology Leukemia, Myeloid, Acute/genetics* Leukemia, Myeloid, Acute/pathology Leukemia, Myeloid, Acute/therapy Male Middle Aged Mutation Myeloproliferative Disorders/etiology Myeloproliferative Disorders/genetics* Myeloproliferative Disorders [ncbi.nlm.nih.gov]
CML has a simpler etiology than most cancers ( 4 ); moreover, its time course is comparatively easy to monitor in the clinic ( 5–7 ). [cancerres.aacrjournals.org]
Epidemiology
[…] or biphasic clinical course as it progresses from a chronic phase to an accelerated phase and on to a terminal blast crisis · In the chronic phase, blasts are less than 2 percent, granulocytes are morphologically normal with no evidence of dysplasia Epidemiology [errolozdalga.com]
[…] cells > 20% blasts in blood or bone marrow samples Adapted from American Cancer Society Blast Crisis Life-threatening hematologic emergency Increase in blast cells in the marrow results in blood hyperviscosity and relative reduction of other cell lines Epidemiology [coreem.net]
Epidemiology The annual incidence of CML is between 1 and 2 cases per 100,000. [ 3 ] CML represents about 15% of all adult leukaemias. [ 1 ] Presentation can occur at any age; however, it is rare in children (CML represents only 5% of childhood leukaemia [patient.info]
Fewer than 1% of patients died because of leukemia progression. [24] Epidemiology [ edit ] United Kingdom [ edit ] CML accounts for 8% of all leukaemias in the UK, and around 680 people were diagnosed with the disease in 2011. [32] United States [ edit [en.wikipedia.org]
Pathophysiology
Hyperleukocytic leukemias and leukostasis: a review of pathophysiology, clinical presentation and management. Leuk Lymphoma. 2000; 39: 1-18 PMID: 10975379 Shet AS et al. Chronic myelogenous leukemia: mechanisms underlying disease progression. [coreem.net]
Pathophysiology: · Myeloproliferative d/o with overproduction of myeloid cells and mature granulocytes (predominantly neutrophils, but also basophils and eosinophils) as well as megakaryocytes and erythroid cells · BCR-ABL fusion (Philadelphia chromosome [errolozdalga.com]
[…] leukaemiachronicmyeloid/symptomsdiagnosis/phases.aspx http://www.cancer.org/cancer/leukemia-chronicmyeloidcml/detailedguide/leukemia-chronic-myeloid-myelogenous-treating-treating-by-phase Further Reading Chronic Myeloid Leukemia Treatment Chronic Myeloid Leukemia Pathophysiology [news-medical.net]
The action of the BCR-ABL protein is the pathophysiologic cause of chronic myelogenous leukemia. [en.wikipedia.org]
Prospects Alternative approaches aim at a better understanding of the pathophysiology of BC and at the recognition of features with prognostic impact. Table 1. [haematologica.org]
Prevention
[…] e18546 Background: Primary goal in management of chronic myeloid leukemia (CML) is to prevent progression to BP-CML. Even with the use of induction chemotherapy with a TKI, outcome remains dismal in BP-CML. [ascopubs.org]
Abstract: P608 Type: Poster Presentation Presentation during EHA22: On Saturday, June 24, 2017 from 17:30 - 19:00 Location: Poster area (Hall 7) Background Primary goal of management in chronic myeloid leukemia (CML) is to prevent disease progression [learningcenter.ehaweb.org]
This illustrates the need to develop predictive models to identify patients early whose response to TKI therapy will not be durable and hopefully prevent the transformation to advanced disease. [ncbi.nlm.nih.gov]
The following chemotherapy drugs may be given directly into the spinal fluid (called intrathecal chemotherapy) to prevent or treat CML in the central nervous system: cytarabine (Cytosar, Ara-C) methotrexate Stem cell transplant An allogeneic stem cell [cancer.ca]