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)

We report a rare occurrence of myeloid sarcoma in a 7 years old child with acute myeloblastic leukaemia (AML-FAB type M2). He presented with fever, generalized weakness, bilateral proptosis and left parotid swelling. CT scan revealed a mass in paranasal sinuses extending into brain and retro-orbital region. Diagnosis of AML M2 was made on bone marrow aspiration and special stains. Induction therapy for AML was given according to standard protocol. The extramedullary lesion as well as the acute leukaemia went into complete remission.
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PMID:Myeloid sarcoma in a child with acute myeloblastic leukaemia. 2171 97

Here we report a 3-year-old boy with myelomastocytic leukemia. The patient presented with fatigue and right eye proptosis. Bone marrow revealed acute myeloid leukemia with t(8;21) and trisomy 8. Induction therapy produced marked reduction in marrow myeloblasts with the emergence of 13% atypical mast cells. These cells were subsequently identified in retrospect in the diagnostic marrow consistent with myelomastocytic leukemia. His clinical course was notable for the difficulty in the eradication of the leukemic process and resembled that of adults with systemic mastocytosis with associated hematologic non-mast cell lineage disease. To the best of our knowledge, this is the youngest individual reported. The implications of mast cell lineage involvement in acute myeloid leukemia are reviewed.
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PMID:Myelomastocytic Leukemia With t(8;21) in a 3-year-old Child. 2204 88

A 49-year-old woman with acute myeloid leukemia, FAB M1 subtype, and 12p deletion, presented with progressive right proptosis and diplopia for 1 week. Orbital CT revealed a homogenously enhancing, orbital mass engulfing the inferior rectus muscle. Histopathology revealed myeloid sarcoma for which she underwent external beam radiotherapy. Subsequently, there was no sign of local recurrence, but she succumbed to leukemia involving the central nervous system. This is the first case, to the authors' knowledge, of an orbital sarcoma of FAB M1 myeloblasts bearing a 12p deletion.
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PMID:Orbital myeloid sarcoma in an adult with acute myeloid leukemia, FAB M1, and 12p-deletion. 2331 98

Myeloid sarcoma is a tumor of immature myeloid cells occurring in many extramedullary sites, orbit being one of them where the tumor may occur prior to or after the diagnosis of underlying disease. We report a case of a 17-year-old male who presented with upper eyelid swelling, proptosis and diplopia after presumed blunt trauma without any other clinical signs and symptoms. Initial imaging suggested possibility of subperiosteal hematoma. Magnetic resonance imaging studies demonstrated a solid tumor. Complete excision of the tumor and histopathologic diagnosis revealed evidence of acute myeloid leukemia (AML). There were no other sites indicating any tumoral process; however, bone marrow aspirate revealed an evidence of systemic disease. After chemotherapy and allogenic bone marrow transplant, the patient had complete remission of his disease. An episode of graft vs host reaction resulting in severe dry eyes along with skin eruptions was treated successfully by immunosuppression and topical lubrication without any visual or systemic sequela. This case emphasizes on the need for an aggressive work-up for any unusual orbital lesion in the absence of any explainable etiology. Further, AML may be suspected in the cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.
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PMID:Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia. 2396 Sep 99

Granulocytic sarcoma is a rare variant of a myeloid malignancy, which shows an extra-medullary tumour mass which is composed of myeloblasts and myeloid precursors with varying degrees of differentiation. It occurs most commonly in bone, periosteum, soft tissue, lymph nodes, and skin; although it can occur anywhere throughout the body. Here, we are reporting two cases of orbital granulocytic sarcoma in children, which presented clinically with proptosis and periorbital swellings, which were first diagnosed by Fine Needle Aspiration Cytology (FNAC). Later, peripheral blood and bone marrow aspirate examinations revealed the evidence of Acute Myeloid Leukaemia (AML). These cases are being documented to demonstrate the utility and diagnostic accuracy of FNAC in evaluation of this entity, in cases of unsuspected AML. Recognition of this rare entity is important, because giving an early aggressive chemotherapy can cause regression of the tumour and thus improve the patient survival.
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PMID:Granulocytic sarcoma presenting as an orbital mass: report of two cases. 2408 83

Five case histories are presented. Waldenstrom's macroglobulinaemia caused bilateral central retinal vein occlusion, proptosis was the presenting feature of retro-orbital plasmacytoma in relapsed multiple myeloma, a red painful eye was due to neovascular glaucoma in primary polycythaemia, bilateral VIth nerve palsy caused convergent squint and diplopia in meningeal relapse of acute lymphoblastic leukaemia and lymphoma of the eyelid caused complete ptosis. Interdisciplinary management is described. Ophthalmological lesions in haematological disease should be promptly recognized and managed. Collaboration between ophthalmology and haematology departments may be effective for palliative management.
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PMID:Ophthalmic manifestations of haematological disorders. 2417 39

Ewing's sarcoma (EWS) is an undifferentiated sarcoma of bone. Its morphologic appearance resembles many other malignant small round cell tumors. Due to the morphologic overlap, there is diagnostic difficulty and for accurate diagnosis, requires special studies such as immunohistochemistry, electron microscopy, cytogenetics, and molecular genetic analysis. We report a case of metastatic EWS from orbital mass in a 14-year-old female child diagnosed by cytology after clinicopathologic evaluation. She presented with low back ache of 1 year followed by proptosis of the right eye and swelling of the right side chest wall. Cytosmear and Tru-cut biopsy was taken from the orbital mass showed features of EWS. It was confirmed later by further studies including demonstration of EWS/friend leukemia integration-1 fusion gene by molecular genetic analysis.
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PMID:Cytodiagnosis of metastatic Ewing's sarcoma of orbital mass and its confirmation by demonstration of EWS/friend leukemia integration 1 fusion gene. 2519 Sep 84

We report the case of a 3-year-old child, who presented with lid swelling which progressed to proptosis of the left eye. He also had systemic symptoms of fatigue and weight loss. An examination revealed hepatosplenomegaly and lymph node enlargement. Investigations showed a peripheral smear with blast cells, which were also revealed through a bone marrow biopsy. A CT scan showed a mass lesion in the left orbit that had infiltrated into the surrounding tissues. He was diagnosed with acute lymphoblastic leukaemia (ALL) with left-sided orbital mass secondary to it. Haematogenous masses in the orbit are commonly due to granulocytic sarcomas, which are usually associated with acute myelogenous leukaemia (AML), not ALL, and are rare especially when they precede systemic disease.
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PMID:Orbital mass secondary to acute lymphoblastic leukaemia in a child: a rare presentation. 2520 76

Simultaneous proptosis and facial palsy as the clinical presentation of childhoodAcute Myeloid Leukaemia (AML) is very rare. To date, no case have beenreported anywhere to the best of our knowledge. Extra medullary leukemic deposits or Granulocytic Sarcoma (GS) is a rare manifestation in about 3% of childhood AML, 9.3% of GS manifested as orbit deposits causing proptosis in one or both eyes. CNS infiltration or acute otomastoiditis subsequent to leukemic infiltration of the temporal bone may be implicated with facial paralysis. We are here with reporting the case in a 3-year-old boy who presented with proptosis and facial palsy in a case of AML. The purpose of reporting this case is to emphasize the need of examining the peripheral blood and bone marrow in children presenting as proptosis and facial palsy for early diagnosis of childhood AML.
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PMID:Proptosis and facial palsy as an unusual clinical presentation of acute myeloid leukemia. 2525 Jan 10

We report bilateral orbital and choroidal involvement as the presenting sign of acute myeloid leukemia in a 2-year-old white girl. The patient presented with painless proptosis and subconjunctival hemorrhage. Ophthalmic examination and magnetic resonance imaging revealed bilateral leukemic infiltrates of the orbits and choroid, with an exudative retinal detachment in the right eye. Bone marrow biopsy confirmed acute myeloid leukemia. Following radiation treatment, chemotherapy, and hematopoietic stem cell transplantation, the patient was doing well 12 months after presentation. Outcomes can be poor, even with treatment; prompt recognition of ophthalmic manifestations of leukemia, including proptosis, choroidal infiltration, and retinal detachment, is necessary.
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PMID:Combined orbital proptosis and exudative retinal detachment as initial manifestations of acute myeloid leukemia. 2648 38


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