Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myeloid sarcoma is a tumor mass with extramedullary growth pattern, composed of myeloblasts or immature myeloid cells. The development of myeloid sarcoma may precede or concur with acute or chronic myeloid leukemia (AML or CML) or other myeloproliferative diseases or myelodysplastic syndromes (MDS). Isolated myeloid sarcoma of the breast is very rare. A case is presented of a 25-year-old, previously healthy woman that presented to our department for a palpable node, 5 x 2 cm in size, in the upper medial quadrant of her left breast. Fine needle aspiration (FNA) produced a sample consisting of medium sized blasts. Additional work-up revealed anemia, thrombocytopenia and leukocytosis, along with atypical blasts detected in peripheral blood and bone marrow smear. Based on the morphology, cytochemical characteristics and immature cell immunophenotype, it was considered a case of acute myeloid leukemia without maturation. In spite of intensive chemotherapy, the patient died within a year of diagnosis. In cases of isolated breast myeloid sarcoma, the diagnosis can be missed if the possibility of myeloid sarcoma is not remembered on differential diagnosis of a breast neoplasm.
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PMID:Myeloid sarcoma involving the breast. 2069 44

Chronic myeloid leukemia (CML) is infrequent in children. The best-known treatment is stem cell transplant. In a country with limited resources like Sudan, such expensive therapy is not available. Alternative approaches are needed to help these children. The tyrosine kinase inhibitor-imatinib-might be an answer to this problem. The objective of this study is to determine the pattern of children with CML, their hematological response to imatinib, and tolerance and side effects to this drug. All patients with confirmed BCR-ABL by polymerase chain reaction (PCR) were included in this study. The relevant data were collected and the patients were started on imatinib. Response to treatment was assessed clinically and hematologically only. Cytogenetics and molecular studies are not available. The average age of the 31 patient evaluated was 8.7 years, 2 patients were less than 1 year, and 5 patients, ie, 16%, were 2 years old or less. Chloroma was observed in 6 (19%) patients. The average of the white blood cell (WBC) count was 206.6 x 10(9)/L and the platelet count average was 523 x 10(9)/L. Two (6.5%) of the 31 patients presented as acute myeloid leukemia (AML). All patients had hematological remission within 2 months. Twenty-three (74%) had a sustained remission over an average follow-up period of 26 months (2-67 months). Six (19%) patients died with AML or sepsis. Side effects to imatinib were infrequent, observed in 4 out of 29 (13.7%) patients, and mild. One patient only needed dose modification. No resistance was observed during this period. CML patients present at an earlier age than in other parts of the world. Imatinib is safe and effective in treating pediatric CML where stem cell transplant in not available. Further cytogenetics are important to monitor response and proper management.
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PMID:Pattern of pediatric chronic myeloid leukemia in Sudan and hematological response to imatinib. 2108 63

Granulocytic sarcoma (GS) usually presents concomitantly with or after the onset of acute myeloid leukemia, blastic phase of chronic myeloid leukemia (CML), or myelodysplastic syndromes. Rarely, it may present even before the onset of overt leukemia and when so, it is often misdiagnosed. We are reporting a case of GS of kidney presenting as an isolated renal mass with normal laboratory investigations including a normal peripheral blood smear. It was initially misdiagnosed as lymphoma as the blasts, in addition to the morphological similarity with lymphoma cells, also showed positive immunohistochemistry for B cell markers. Based on further investigations including immunophenotyping and cytogenetic studies, a final diagnosis of CML-blast crisis (mixed phenotype) presenting initially as GS was made. To the best of our knowledge, this is the first antemortem report of nonleukemic GS presenting as kidney mass that later on progressed to CML-blast crisis with mixed phenotype blasts.
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PMID:Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: a diagnostic dilemma. 2193 35

Myeloid sarcoma (MyS) is defined as an extramedullary tumor-forming neoplasm consisting of immature myeloid cells with/without maturation. We experienced a case involving a 68-year-old Japanese male patient who had been followed-up for four years with a diagnosis of chronic idiopathic myelofibrosis/primary myelofibrosis (PMF) and noticed a painful mass in his left axilla. A wedge biopsy characterized the lesion as MyS that displayed megakaryoblastic/megakaryocytic differentiation. As his complete blood count included a few myeloid blasts (1% of WBC) and a bone marrow biopsy detected fibrosis without evidence of acute myelogenous leukemia (AML), a diagnosis of extramedullary blastic transformation of PMF was made, which was confirmed later by V617F mutation in Janus kinase-2 in both initial bone marrow biopsy and axillary tumor biopsy specimens. The patient died of pneumonia eight months after developing the axillary tumor. At autopsy, multiple MyS masses were detected in his soft tissue, but his bone marrow only contained fibrosis. Although MyS rarely develops before the leukemic transformation of PMF, no evidence of AML could be found in the patient's bone marrow at any point during the course of his disease. Thus, it is possible that the blasts in his peripheral blood were derived from the remaining MyS. Furthermore, the present case indicates that extramedullary blastic transformation, which is occasionally seen in CML, can also occur in PMF. Therefore, it is important to recognize that there is a wide variation in the pathogeneses of MyS and PMF.
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PMID:Autopsy case of primary myelofibrosis in which myeloid sarcoma was the initial manifestation of tumor progression. 2261 14

Myeloid sarcoma is a rare tumor of immature myeloid cells in an extramedullary site. Myeloid sarcoma may present in a variety of locations; skin is one of the common sites. It may precede or occur concurrently with acute myeloid leukemia, chronic myeloid leukemia, other forms of myeloproliferative disorders/myelodysplastic syndrome or de novo. We report a case of a 4-month-old female who presented with cutaneous lesions without evidence of leukemia, determined to be de novo myeloid sarcoma. She had erythematous nodules in multiple skin sites. Biopsy revealed a diffuse atypical mononuclear cell infiltrate involving the entire dermis and extending to the subcutis. The infiltrate was diffusely positive for lysozyme, CD43, CD15, CD33, CD68 and CD117 and was negative for CD3, CD20, CD34, CD56, CD79a, CD99, myeloperoxidase, desmin, chromogranin and synaptophysin, supporting a diagnosis of myeloid sarcoma. No leukemic involvement was found on evaluation of peripheral blood or bone marrow aspiration. Chromosomal abnormalities were found at chromosomes 7, 10 and 11. The skin lesions resolved following multiple chemotherapy courses, then recurred requiring additional treatment. De novo myeloid sarcoma involving skin without evidence of leukemia can occur in an infant and may present a diagnostic challenge.
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PMID:De novo myeloid sarcoma in a 4-month-old infant: a case report and review of the literature. 2309 17

Chloroma, also called granulocytic sarcoma or myeloid sarcoma, is a rare malignant extramedullary neoplasm of myeloid precursor cells that occurs usually in association with acute myeloid leukaemia or blast phase of chronic myeloid leukaemia (CML). The chloromas are regarded as an early herald of a systemic relapse, rather than as a localised process. The author reports a rare case of multiple skin chloromas in the chronic phase of CML. The patient was treated initially with hydroxyurea and chloromas disappeared. Patient has been followed for around three and half years and maintained in chronic stable phase on imatinib therapy.
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PMID:Multiple skin chloromas: a rare presentation of chronic myelogenous leukaemia in chronic stable phase. 2359 14

Granulocytic sarcoma also called myeloid sarcoma is an extramedullary tumor of immature granulocytic cells. It is a rare entity, and mostly accompanied by acute myeloid leukemia. It is observed during the course of myeloproliferative disorders especially in chronic myeloid leukemia and myelodysplastic syndromes. In some rare circumstances, it is detected before clinical signs of leukemia or other diseases. When the bone marrow biopsy reveals no other hematologic malignancies, the granulocytic sarcoma is described as nonleukemic, primary or isolated. It is observed at any part of the body but the most common locations are soft tissues, bone, peritoneum and lymph nodes. Presenting signs or symptoms are mainly due to mass effect of the tumor and dysfunction of the organ, or the tissue that is affected. The diagnosis is performed by biopsy of the tumor. The tumor consists of immature granulocytic cells, which could be documented by H&E, immunohistochemistry, and flow cytometric methods. Fluorescence in-situ hybridization and molecular analysis are also performed. The optimal time and type of treatment is not clear. Surgery could be an option especially for tumors, which cause organ dysfunction and/or obstruction. Systemic treatment should be considered in all patients because without systemic treatment, relapses and progression to acute myeloid leukemia is the ultimate fate of the disease in many cases. Cytarabine-containing remission-induction chemotherapies have been the most applied therapeutic strategies, but it is not clear whether the consolidation therapies are required or not, and what kind of regimens are appropriate. The role of hematopoietic stem cell transplantation (HSC) as a consolidation regimen is not clear, but, after the relapse of the disease with or without bone marrow involvement, HSC transplantation should be considered in suitable patients after the reinduction performed by AML chemotherapies. There is only limited data about the role of radiotherapy in these patients. It could be used in patients with relapsed disease, organ dysfunction which should be quickly relieved and inadequate response to chemotherapy. The effect of radiotherapy on overall survival is not known. New prospective studies and clinical trials are needed to generate guidelines for the treatment of primary granulocytic sarcomas.
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PMID:Granulocytic sarcoma: a systematic review. 2439 4

Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin.
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PMID:Cutaneous myeloid sarcoma of the penile foreskin. 2491

Myeloid sarcoma is an extramedullary tumor composed of immature myeloid cells that efface the underlying tissue architecture. It is usually associated with acute myelogenous leukemia, but can be associated with myeloproliferative neoplasms, myelodysplastic disorders, or myeloproliferative/ myelodysplastic syndromes. If it is unrecognized, appropriate chemotherapy may be delayed and survival jeopardized. We present a case of a myeloid sarcoma presenting in an otherwise asymptomatic patient who ultimately was found to have chronic myelogenous leukemia, presenting in blast crisis. We also review the distinct clinical and pathologic features of myeloid sarcoma, as well as treatment of the disease.
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PMID:Myeloid sarcoma as the presenting symptom of chronic myelogenous leukemia blast crisis. 2498 78

Granulocytic sarcoma occurs most commonly in acute myelogenous leukemia. The appearance of granulocytic sarcoma in chronic myelogenous leukemia signals accelerated phase/ blast transformation. This is a rare case of undiagnosed chronic myelogenous leukemia with granulocytic sarcoma causing cord compression, which went into tumour lysis syndrome requiring dialysis after starting of steroids and radiotherapy. A 43-year-old male presented in emergency department with acute onset of flaccid paralysis. On clinical examination, there was hepatosplenomegaly and lower motor neuron paralysis in the lower limbs. The peripheral smear was consistent with chronic myelogenous leukemia in chronic phase. The MRI spine revealed para-spinal and epidural masses causing cord compression and the biopsy from the paraspinal mass was consistent with granulocytic sarcoma.
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PMID:Granulocytic sarcoma with compressive myelopathy: a rare presentation of chronic myelogenous leukemia. 2517 19


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