Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023418 (leukemia)
 93,477 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acute Myelogenous Leukemia (AML) typically involves intramedullary proliferation of myeloid precursor cells. Extramedullary manifestations of AML are exceedingly rare, but do occur. Granu-locytic sarcoma, or chloroma, is one example of extramedullary leukemia cells forming a tumorous mass. We report a case of Chloroma in the volar forearm compartment presenting with both median nerve compressive neuropathy and apparent tenosynovitis. Abscess was at the top of the early differential, and the patient was scheduled for operative debridement. However, further evaluation indicated that chloroma was present, thus obviating the need for emergent surgical intervention and necessitating the induction of chemotherapy. To our knowledge this is the first report of chloroma in this location and with these presenting symptoms.
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PMID:Chloroma of the forearm: a case report of leukemia recurrence presenting with compression neuropathy and tenosynovitis. 1974 97

Granulocytic sarcoma (GS) is composed of immature granulocytic precursors and is usually found in acute myeloid leukemia (AML) patients with t(8;21). Inv(16) is rarely associated with GS comparing with t(8;21) leukemia. Here we describe an abdominal GS patient in AML-M2 with acquired translocation between chromosomes 6 and 17 and inv (16). We have also summarized 20 reported GS cases with inv(16) and found that chloroma was most often found in abdominal lesions. Intestine maybe a tissue specific target for the expression of inv(16) leukemia. Complete physical examination and molecular diagnosis are necessary for AML patients to benefit from the diagnosis and therapeutic strategy.
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PMID:Granulocytic sarcoma of abdomen in acute myeloid leukemia patient with inv(16) and t(6;17) abnormal chromosome: case report and review of literature. 2011 51

Paraplegia due to a spinal cord epidural mass is an extremely rare presentation of undiagnosed leukemia. We are reporting a case of 14-year-old girl, who presented with paraplegia due to thoracic epidural mass, as the initial presenting manifestation of acute myeloid leukemia. Granulocytic sarcoma or chloroma should be considered in the differential diagnosis of an epidural mass in patients with or with out leukemia granulocytic sarcoma, which are rare extramedullary tumor-like proliferation of myelogenous precursor cells that may de novo precede acute leukemia or coincide with the first manifestation or relapse of acute myeloid leukemia.
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PMID:Unusual case of paraplegia. 2017 2

This article gives an overview of the most common and clinically relevant tumors of the orbit and their treatment. The most common orbital tumors in childhood are cystic tumors, such as dermoid and epidermoid cysts. Capillary hemangiomas are the most common primary benign tumors of the orbit and appear mostly in the first year of life. In contrast teratomas are rare and histologically mostly benign. Neural tumors are, for example, gliomas of the optic nerve and in 25-50% of cases are associated with neurofibromatosis. Rhabdomyosarcoma is a rapidly growing malignant orbital tumor whereby the stage and localization are the most important prognostic factors for survival chance in children. Leukemia can be associated with a chloroma especially in the first decade of life. Lymphoproliferative diseases, vascular and cystic tumors in particular are known as tumors of adulthood. In addition to fibroosseous and mesenchymal tumors, neural forms, such as schwannomas are also important. Secondary tumors of the orbit are often manifested in the nose and paranasal sinuses.
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PMID:[Tumors and tumor-like lesions of the orbit]. 2060 7

Acute myeloid leukemia (AML) can affect not only bone marrow (BM) and peripheral blood (PB), but also the compartment of cerebrospinal fluid (CSF). Besides standard chemotherapy, specific and non-specific immunotherapy has been employed synergistically to treat AML patients. Here we report on a patient who received standard chemotherapy, unspecific immunotherapy with interleukin-2, as well as later specific CD8+ T-cell stimulation by RHAMM-R3 peptide vaccination. The patient maintained a complete remission in BM and PB, while he developed recurrent relapses in the CSF. Moreover, the patient developed a chloroma in the vicinity of neuronal sheaths during hematological CR, but high leukemia cell numbers within the CSF spaces over a long time period. This rare observation demonstrates several aspects. There is a previously unknown site of leukemia cell distribution, namely the peripheral cerebrospinal outflow pathway (PCOP). This demonstrates the ineffective therapy of this previously unknown mechanism of leukemia cell spread. The hypothesis that the PCOP is a site of physiological CSF-T-cell trafficking, and the lumen of the PCOP should be considered as an extension of the subarachnoidal spaces without closed anatomical borders is supported by this observation. The CSF spaces, but possibly specifically the PCOP, may represent a previously unknown survival niche of tumor cells within intrathecal spaces.
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PMID:Spreading of acute myeloid leukemia cells by trafficking along the peripheral outflow pathway of cerebrospinal fluid. 2173 62

Extramedullary (EM) manifestations of acute leukemia include a wide variety of clinically significant phenomena that often pose therapeutic dilemmas. Myeloid sarcoma (MS) and leukemia cutis (LC) represent 2 well-known EM manifestations with a range of clinical presentations. MS (also known as granulocytic sarcoma or chloroma) is a rare EM tumor of immature myeloid cells. LC specifically refers to the infiltration of the epidermis, dermis, or subcutis by neoplastic leukocytes (leukemia cells), resulting in clinically identifiable cutaneous lesions. The molecular mechanisms underlying EM involvement are not well defined, but recent immunophenotyping, cytogenetic, and molecular analysis are beginning to provide some understanding. Certain cytogenetic abnormalities are associated with increased risk of EM involvement, potentially through altering tissue-homing pathways. The prognostic significance of EM involvement is not fully understood. Therefore, it has been difficult to define the optimal treatment of patients with MS or LC. The timing of EM development at presentation versus relapse, involvement of the marrow, and AML risk classification help to determine our approach to treatment of EM disease.
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PMID:How I treat extramedullary acute myeloid leukemia. 2179 42

The most frequent initial ocular manifestation of acute myeloid leukaemia (AML) is retinal involvement. Here, we report an unusual case of AML associated with a pontine chloroma presenting with gaze palsy as the initial symptom. A 77-year-old Caucasian man presented to the Eye Casualty complaining of a one-day history of blurred vision. On examination, his face was turned to the left, both eyes were fixed in dextroversion and the patient demonstrated left gaze palsy associated with left motor neurone VII palsy. Baseline blood investigations revealed leucocytosis with 60% circulating myeloblasts. A bone marrow biopsy confirmed the diagnosis of myelomonocytic leukaemia. A CT scan showed a well-circumscribed lesion in the dorsal pons, most likely representing a chloroma. Chloromas or myeloblastomas related to AML are localised extramedullary tumours composed of leukaemic myeloid cells. Chloromas may be present at the time of the initial diagnosis of leukaemia or may precede the diagnosis by 1 month to 2 years; however, their occurrence in the central nervous system is rare, comprising 1-6% of all chloromas. This case illustrates the many different ways that AML can manifest itself in the eyes, and ophthalmologists should be aware of the great variety of presenting symptoms in undiagnosed AML.
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PMID:Acute myeloid leukaemia presenting as gaze palsy. 2217 4

Some oncolytic viruses, such as myxoma virus (MYXV), can selectively target malignant hematopoietic cells, while sparing normal hematopoietic cells. This capacity for discrimination creates an opportunity to use oncolytic viruses as ex vivo purging agents of autologous hematopoietic cell grafts in patients with hematologic malignancies. However, the mechanisms by which oncolytic viruses select malignant hematopoietic cells are poorly understood. In this study, we investigated how MYXV specifically targets human AML cells. MYXV prevented chloroma formation and bone marrow engraftment of two human AML cell lines, KG-1 and THP-1. The reduction in human leukemia engraftment after ex vivo MYXV treatment was dose-dependent and required a minimum MOI of 3. Both AML cell lines demonstrated MYXV binding to leukemia cell membranes following co-incubation: however, evidence of productive MYXV infection was observed only in THP-1 cells. This observation, that KG-1 can be targeted in vivo even in the absence of in vitro permissive viral infection, contrasts with the current understanding of oncolytic virotherapy, which assumes that virus infection and productive replication is a requirement. Preventing MYXV binding to AML cells with heparin abrogated the purging capacity of MYXV, indicating that binding of infectious virus particles is a necessary step for effective viral oncolysis. Our results challenge the current dogma of oncolytic virotherapy and show that in vitro permissiveness to an oncolytic virus is not necessarily an accurate predictor of oncolytic potency in vivo.
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PMID:Acute myeloid leukemia targeting by myxoma virus in vivo depends on cell binding but not permissiveness to infection in vitro. 2234 1

Intracranial occurrence of a chloroma (myeloid sarcoma, MS) in the absence of a preceding hematological malignancy is unusual. We report the case of a 20-year-old man who presented with Garcin's syndrome of short duration. MRI revealed multiple extra-axial contrast enhancing lesions: two mirror lesions on the skull base, and one in the right parietal convexity. The parietal lesion was excised and histologically and immunohistochemically proved to be a differentiated variant of MS. Peripheral blood smear and bone marrow biopsy ruled out an underlying leukemia or myeloproliferative disorder. With a diagnosis of intracranial de-novo MS, he was referred for chemotherapy and radiation therapy. 15 months later, his clinical status remained the same while his imaging showed marginal decrease in size of the lesions. A repeat bone marrow biopsy remained normal. This is a first-of-its- kind report of multiple intracranial lesions of a de-novo MS presenting as Garcin's syndrome. Radiological differentials, immunohistochemical variants and management options related to MS are discussed in the light of the reported case.
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PMID:Multiple intracranial de-novo chloromas presenting with Garcin's syndrome. 2254 80

Granulocytic sarcoma (GS) is a rare tumor consisting of immature cells of granulocytic lineage. It is also called chloroma, referring to the green color of the tumor caused by high levels of myeloperoxidase in the cells. GS is often associated with acute myeloblastic leukemia. We report the case of a 4-year-old boy with a diagnosis of acute myeloblastic leukemia. Abdominal ultrasonography demonstrated a solid, vascularized, heterogeneous, polypoid formation involving the posterior wall of the bladder. Further studies confirmed the etiology of the tumor. On control ultrasonography, a marked decrease in tumor size 15 days after treatment was revealed. We describe the imaging findings in this patient and review the literature about this infrequent entity. Although imaging findings are not specific and considering that extramedullary involvement in leukemia is very sensitive to treatment, inclusion of GS in the differential diagnosis and awareness of the possible sites of occurrence and imaging features is essential to avoid unnecessary therapeutic measures.
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PMID:Granulocytic sarcoma of the urinary bladder in a pediatric patient. 2276 4


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