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Blast Phase

Blast Crises


Presentation

  • 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]
  • Sixty-seven percent of patients presented with myeloid immunophenotype and 29% presented with a lymphoid immunophenotype.[healio.com]
  • Surprisingly, no extramedullary blast crises of B- lymphoid lineage have been reported before among cases of CML as the initial presentation.[mattioli1885journals.com]
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]
Fever
  • 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]
  • 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]
  • When tiredness, fever, and an enlarged spleen occur during the blastic phase, it is called blast crisis.[en.wikipedia.org]
  • Ten months later, he presented with fevers and leukocytosis (WBC, 109.7 10 9 /L), with 20% blasts. The blasts were moderate in size and had fine chromatin and a moderate amount of granular cytoplasm (panel B).[bloodjournal.org]
Anemia
  • At BP, 63% of patients had leukocytosis (white blood cell count 10 x 10(9)/L), 74% had anemia (hemoglobin value (c) 2009 Wiley-Liss, Inc.[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]
  • These cells eventually spill into the blood and other organs, causing symptoms such as fatigue from anemia or an enlarged spleen.[texasoncology.com]
Weight Loss
  • The effect is similar to that of acute leukemia, and includes symptoms such as weight loss, night sweats, and bone pain.[disabilitybenefitscenter.org]
  • 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]
  • 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]
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]
  • The blast phase of Chronic Myelogenous Leukemia (CML) can also bring about fever, fatigue, tiredness, and an enlargement of the spleen. When this happens, it is called a blast crisis.[disabilitybenefitscenter.org]
  • In this study, we present a JAK2 V617F positive MPN patient who experienced fatigue and splenomegaly, transforming into JAK2 V617F negative AML. A diagnosis of acute monocytic leukemia was made in MPN blast phase.[journals.lww.com]
  • […] set forth by the European LeukemiaNet, recommending 30% blasts in the blood or bone marrow or the presence of extramedullary disease. 1 Clinically, blast phase CML may present with constitutional symptoms, bone pain, or symptoms related to cytopenias (fatigue[mdedge.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]
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]
  • Clinical findings: Individuals with CML present with splenomegaly (enlarged spleen); elevated white blood counts exceeding 300,000- 600,000 cells/ μL; and retinas showings papilledema, venous obstruction, and hemorrhages.[secure.ssa.gov]
  • Given the presence of constitutional symptoms and massive splenomegaly, the patient inquired about treatment with the JAK inhibitor ruxolitinib.[bloodjournal.org]
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]
  • Given the presence of constitutional symptoms and massive splenomegaly, the patient inquired about treatment with the JAK inhibitor ruxolitinib.[bloodjournal.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]
Abdominal Pain
  • pain, apathy, confusion, extreme weakness, and hypotension; if untreated these progress to shock and then death. aplastic crisis a sickle cell crisis in which there is temporary bone marrow aplasia. catathymic crisis an isolated, nonrepetitive act of[medical-dictionary.thefreedictionary.com]
  • pain due to splenic enlargement or infarct, loss of well-being, unexplained weight loss, extramedullary enlarging masses, arthralgia not responding to treatment, and rarely CNS infarct (as observed in our case).[ 3 ] In a study by Alwan[ 2 ] on 53 patients[ncbi.nlm.nih.gov]
  • 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]
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]
Headache
  • 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]
  • 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]

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

  • The best prognosis was found in the myeloid group, followed by the lymphoid, myelomonocytic, megakaryoblastic and finally the promyelocytic groups, in this order.[ncbi.nlm.nih.gov]

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]
Sex distribution
Age distribution

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]
  • References 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

  • 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]
  • […] 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]
  • 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]

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