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)

A 16-year-old girl who had been treated for B-precursor acute lymphoblastic leukemia (ALL) presented with a mass in the left orbit as an isolated relapse. Five years earlier, she had undergone chemotherapy that promptly resulted in a complete remission. For prophylaxis of central nervons system leukemia, the patient received cranial radiotherapy (18 Gy) with shielding of the orbit and 15 cycles of intrathecal MTX and prednisolone. Cerebrospinal fluid analysis did not detect infiltration during the treatment course. Exophthalmos of the eft eye and hyperemia of the conjuctiva developed 15 months after therapy. Computed tomography showed a retro-orbital mass behind the left eye. Though bone marrow aspiration was negative, biopsy specimens from the mass disclosed the infiltration of CD10-positive leukemic blasts. After 8 courses of chemotherapy, 5 cycles of intrathecal MTX, and orbital irradiation (24 Gy), the mass disappeared. All relapse as an isolated retro-orbital mass is very rare, and only 3 cases have been reported to date.
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PMID:[Relapse of acute lymphoblastic leukemia as a retro-orbital mass]. 1062 31

We would like to report the use of liposomal amphotericin in eradicating mucormycosis in two patients who had relapsed acute leukaemia. The first patient with relapsed acute myeloid leukaemia developed a rapidly expanding solitary necrotic neck lesion associated with opacity of maxilliary sinus at a time when he was profoundly pancytopenic following high dose chemotherapy. The second patient was a 3-year-old boy with pre-B acute lymphoblastic leukaemia who developed a central nervous system relapse whilst on his first line treatment and was treated with more aggressive chemotherapy on the Medical Research Council Relapse Protocol. During a period of profound pancytopenia following re-induction therapy, including high dose steroids and prolonged course of antibiotics for proven septicaemia, he developed periorbital swelling and proptosis and a clinical diagnosis of rhinocerebral mucormycosis was made. Both patients were treated with high doses of liposomal amphotericin (Ambisome Nexstar). The doses were escalated to 10 and 15 mg/kg/day, resulting in successful eradication of the mucormycosis.
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PMID:Zygomycosis in relapsed acute leukaemia. 1112 Jun 17

Acute fulminant fungal sinusitis is characterized by acute symptoms and rapid progress with a mortality rate of 60-80%. A large number of survivors have permanent neurological, visual and cosmetic disabilities. This clearly underscores the need of early recognition of this disease in at risk population in order to start urgent treatment. The at-risk population of diabetics, AIDS and other immunosuppressed is likely to increase, as will the incidence of acute fulminant fungal sinusitis. In the present study we have reviewed nine cases of acute fulminant fungal sinusitis to determine clinical presentation, related radiological picture and optimum treatment. Most common presenting features were fever, headache, facial swelling and proptosis. Many patients presented with blindness, facial paralysis and meningitis. Predisposing causes were uncontrolled diabetes with ketoacidosis in four out of six cases, post renal transplant immunosuppression and leukemia. All patients were treated with amphotericin B or liposomal amphotericin B (AmBisome). Diagnosis was confirmed by biopsy and culture of sinus mucosa, soft tissues of cheek, or orbit. Mucor (Zygomycetes) was identified on culture or histopathology in all cases. Surgical debridement was performed in seven cases. Six out of nine patients survived but morbidity was high: only two patients survived without any permanent disability.
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PMID:Acute fulminant fungal sinusitis: clinical presentation, radiological findings and treatment. 1160 98

A 19-year old girl suffered from relapse of chronic myeloid leukemia (CML) after bone marrow transplantation. The disease was controlled by interferon and imatinib mesylate, but was complicated by autoimmune hyperthyroidism. She presented with unilateral proptosis with no extraocular muscle or visual defect at 26 months follow-up. Systemic investigations showed no recurrence of leukemia or thyrotoxicosis. Magnetic resonance imaging revealed an extensive retro-orbital base of skull lesion. A trans-oral biopsy showed fibrous dysplasia and continuous observation was advised. The unusual sequence of events and the differential diagnoses for unilateral proptosis in post bone marrow transplantation (BMT) cases are discussed.
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PMID:Fibrous dysplasia masquerading as extramedullary relapse after bone marrow transplantation for chronic myeloid leukemia. 1469 42

Two cases of granulocytic sarcoma presenting as childhood proptosis are described. This highlights the significance of peripheral blood smear examination in establishing the diagnosis of Acute Myeloid Leukaemia (AML).
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PMID:Childhood proptosis: the invaluable but overlooked peripheral blood smear. 1588 19

Orbital granulocytic sarcoma is a localized tumor consisting of malignant cells of myeloid origin. This tumor may present in association with acute myelogenous leukemia. Granulocytic sarcoma may be found in a variety of locations throughout the body including the orbit and typically affects children and young adults. There is a slight male predominance in these cases. This is an uncommon case report of a 33-year-old Latin-American woman who was admitted to the Hospital for rapidly progressive orbital proptosis. There was no systemic manifestation of leukemia. The occurrence of orbital granulocytic sarcoma before the development of systemic leukemia in children and young adults is not uncommon and these cases frequently develop hematological evidence within 2 months after initial orbital disease. In this case report, there was no systemic manifestation of leukemia in the last 30 months, even in the presence of orbital tumors. Granulocytic sarcoma is most frequently confused with malignant lymphoma, rhabdomyosarcoma and neuroblastoma. The differential diagnosis of these cases can be challenging, particularly when there is no evidence of systemic leukemia, when imaging features are not sufficiently specific to distinguish granulocytic neoplasms from other tumors. To establish the diagnosis often a biopsy is required. The treatment in such cases (orbital granulocytic sarcoma) is not standardized. Orbital granulocytic sarcoma may be suspected in cases of orbital tumors even in the absence of systemic manifestations of leukemia at any age.
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PMID:[Orbital granulocytic sarcoma: case report]. 1632 47

To the best of our knowledge, ocular leukemia has not been reported in Saudi Arabia. Seventy-two leukemia patients were seen in King Abdulaziz University Hospital in Jeddah over the last 10 years. Thirty-one of those patients had an ocular examination. Seventeen of these were found to have ocular abnormalities related to leukemia. Ten had acute lymphoblastic leukemia, four had acute myeloid leukemia and three had chronic myeloid leukemia. The retina and vitreous were involved in 12 of the ocular relapses, the anterior segment leukemic infiltrate in nine, glaucoma in five, opportunistic infection in four, cranial nerve palsies in three, proptosis and hypotony in two. Conjunctival hemorrhage, choroidal infiltrate and cataract were present in one relapse each. The optic nerve was involved in nine relapses, which is a serious condition that requires immediate intervention to save the patient's sight. Computed tomography (CT) scan of the brain and orbit was useful in differentiating between optic nerve infiltrate and papilledema. Most ocular relapses responded well to chemotherapy except relapses with optic nerve and anterior segment infiltrate, which required irradiation to save the patient's sight and prevent further relapses. In conclusion, the presented data have shown that ocular leukemia is not rare and emphasizes the importance of early ophthalmologic examination and radiation.
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PMID:Ocular leukemia in King Abdulaziz University Hospital - Jeddah. 1759 May 72

Here we report a case of invasive pansinusitis with proptosis of the right eye caused by Aspergillus flavus in an immunocompromised patient with acute biphenotypic leukemia without aggressive therapy response.
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PMID:Invasive Aspergillus flavus sinusitis: case report in a patient with biphenotypic acute leukemia. 1922 93

Emergencies in childhood orbital tumorals are rare. The absolute emergency involves malignant primary orbital tumors, such as rhabdomyosarcoma or secondary malignant tumors (metastatic neuroblastoma, leukemia), involving a vital prognosis requiring prompt diagnosis. Delayed emergencies are usually vascular lesions. Among these lesions, immature orbital hemangioma, with a good prognosis, must be distinguished from orbital adnexal lymphangiomas, which are less frequent but can lead to dramatic cosmetic and functional disorders. In rare cases, they can be responsible for sudden, painful proptosis, due to orbital hemorrhage, with a risk of optic nerve compression, requiring emergency surgical treatment. Neurogenous lesions, either isolated, such as in gliomas, or associated with a systemic disease, such as Recklinghausen neurofibromatosis, threaten the functional prognosis. Diagnosis of pediatric orbital tumors is based on a good clinical examination, precise imaging investigations, and evaluation of the locoregional extension of the tumor. Biopsy is required in emergency situations, when rhabdomyosarcoma is suspected, in order to start the chemotherapy. However, the biopsy can be superfluous, and even useless or dangerous, when clinical and imaging investigations are sufficient to provide a diagnosis of capillary hemangioma, lymphangioma, or metastatic tumor from an abdominal malignancy. Treatment is closely related to the etiopathogenesis of the tumor. The outcomes are vital, functional and cosmetic. They may require orbital surgery (biopsy, tumoral resection, orbital decompression in case of a compressive hemorrhage), systemic corticotherapy (as in immature adnexal hemangioma), radiation, and chemotherapy (rhabdomyosarcoma, secondary malignant tumor). These diseases require a pediatric ophthalmological medical center specializing in orbital surgery, with close collaboration of multiple specialists such as onco-pediatricians and neurosurgeons.
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PMID:[Orbital tumor emergencies in childhood]. 1976 75

Leukemias are the commonest childhood malignancy in West Bengal. This study was undertaken on 75 children at NRS Medical College, West Bengal to determine the distribution of signs and symptoms of leukemia and to identify unusual clinical features. After obtaining clinical history, physical examination, hematological and radiological investigations were performed. Acute lymphoblastic leukaemia (ALL, 72%) was the commonest followed by acute myeloid leukaemia (AML, 18.7%). Common symptoms and signs were fever (85.3%), pallor (64%), hepatomegaly (72%), splenomegaly (60%) and lymphadenopathy (50.7%). The uncommon signs and symptoms were abdominal pain (9.3%), joint pain (9.3%), hematemesis and malena (8%), diarrhea (5.3%), proptosis (2 cases), dysphagia, mediastinal mass and parotid swelling (1 case each). Uncommon clinical presentations lead to delay in diagnosis in some cases. Awareness of uncommon signs and symptoms of childhood leukemia together with laboratory tests may help in earlier diagnosis and proper management of the patients.
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PMID:Childhood acute leukemia in West Bengal, India with an emphasis on uncommon clinical features. 2059 69


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