Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-three children diagnosed with primary myelodysplastic syndrome (MDS) in a single institution over an 8 year period were evaluated with special emphasis on children who presented with extramedullary disease (EMD). EMD was present at diagnosis in 12 (36%) of the 33 children with MDS. Three patients with juvenile myelomonocytic leukemia (JMML) and 2 patients with chronic myelomonocytic leukemia (CMML) presented with pleural effusion. Pericardial effusion was present in 3 of these patients, two of whom also had thrombosis. Pyoderma gangrenosum, relapsing polychondritis were the initial findings in another two cases with JMML. Lymphadenopathy (n=1), gingival hypertrophy (n=2), orbital granulocytic sarcoma (n=1) and spinal mass (n=1) were the presenting findings in 5 patients with refractory anemia with excess of blasts in transformation. Since high-dose methylprednisolone (HDMP, 20-30 mg/kg/day) has been shown to induce differentiation and apoptosis of myeloid leukemic cells in children with different morphological subtypes of acute myeloid leukemia in vivo and in vitro, 25 children with de novo MDS were treated with combined HDMP and cytotoxic chemotherapy. Dramatic improvement of EMD and decrease in blast cells both in the peripheral blood and bone marrow were obtained following administration of short-course HDMP treatment alone as observed in children with AML. HDMP, combined with low-dose cytosine arabinoside and mitoxantrone were used for the remission induction. Remission was achieved in 8 (80%) of 10 children who presented with EMD and in 9 (60%) of 15 children without EMD. Long-term remission (>6 years) was obtained in 4 (two with JMML and two with CMML), three of whom presented with EMD. In conclusion EMD can be a presenting finding in childhood MDS as observed in adults. In addition, the beneficial effect of HDMP combined with more intensive chemotherapy should be explored as alternative therapy in children with MDS not suitable for bone marrow transplantation.
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PMID:Evaluation of children with myelodysplastic syndrome: importance of extramedullary disease as a presenting symptom. 1169 96

MRSA infection or colonization developed in eleven patients with neoplastic disease including malignant lymphoma (5 cases), soft tissue sarcoma (2 cases), acute myeloblastic leukemia (one), myelodysplastic syndrome (one), multiple myeloma (one), and mesothelioma (one) at our ward from October to December 1999. The infections were pneumonia (six cases), enteritis (three), bacteremia (one), and wound infection (one). Ten of 11 cases received antimicrobial agent (s) during one month before isolation of MRSA, suggesting selection of MRSA. Five cases improved and survived, but six cases died of infection. At the isolation of MRSA, the neutrophil count (NC) of the alive cases was 1, 500/microliter or more but the NC of five cases who died was less than 1,000/microliter, especially less than 100/microliter in three cases who had just received a cancer chemotherapy. Pulsed-field gel electrophoresis, performed in 9 cases, showed an identical DNA-pattern of MRSA in 7 cases, indicating a nosocomial infection. Our method to prevent spread of MRSA targeting solely the patients with MRSA infection was obviously unsatisfactory. We should target also the cases of MRSA colonization and make an effort to wash hands more vigorously. Furthermore, radical reformation such as increasing single sick-rooms drastically and increasing the number of nursing staff is also required.
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PMID:[Outbreak of methicillin-resistant Staphylococcus aureus (MRSA) infection or colonization among patients with neoplastic disease: a clinico-epidemiological study of 11 cases]. 1176 76

Second malignant neoplasms are a serious complication after successful treatment of childhood acute lymphoblastic leukemia (ALL). With improvement in survival, it is important to assess the impact of contemporary risk-based therapies on second neoplasms in ALL survivors. A cohort of 8831 children diagnosed with ALL and enrolled on Children's Cancer Group therapeutic protocols between 1983 and 1995 were observed to determine the incidence of second neoplasms and associated risk factors. The median age at diagnosis of ALL was 4.7 years. The cohort had accrued 54 883 person-years of follow-up. Sixty-three patients developed second neoplasms, including solid, nonhematopoietic tumors (n = 39: brain tumors n = 19, other solid tumors n = 20), myeloid leukemia or myelodysplasia (n = 16), and lymphoma (n = 8). The cumulative incidence of any second neoplasm was 1.18% at 10 years (95% confidence interval, 0.8%-1.5%), representing a 7.2-fold increased risk compared with the general population. The risk was increased significantly for acute myeloid leukemia (standardized incidence ratio [SIR] 52.3), non-Hodgkin lymphoma (SIR 8.3), parotid gland tumors (SIR 33.4), thyroid cancer (SIR 13.3), brain tumors (SIR 10.1), and soft tissue sarcoma (SIR 9.1). Multivariate analysis revealed female sex (relative risk [RR] 1.8), radiation to the craniospinal axis (RR 1.6), and relapse of primary disease (RR 3.5) to be independently associated with increased risk of all second neoplasms. Risk of second neoplasms increased with radiation dose (1800 cGy RR 1.5; 2400 cGy RR 3.9). Actuarial survival at 10 years from diagnosis of second neoplasms was 39%. Follow-up of this large cohort that was treated with contemporary risk-based therapy showed that the incidence of second neoplasms remains low after diagnosis of childhood ALL.
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PMID:Low incidence of second neoplasms among children diagnosed with acute lymphoblastic leukemia after 1983. 1203 51

PURPOSE. To report an elderly patient with a primary myelodysplastic syndrome (MDS) type refractory anemia with excess of blasts (RAEB) who developed an orbital granulocytic sarcoma (GS) as a harbinger of an acute myelogenous leukemia (AML). METHODS. A 77-year-old man was diagnosed as having a MDS type RAEB. Eight months later he developed a progressive painless proptosis in his left orbit. A computed tomography (CT) scan revealed a large irregular mass involving the orbit. It showed heterogeneous soft tissue density and no osseous cortical destruction was observed. A diagnostic lateral orbitotomy with an excisional biopsy were performed. RESULTS. Histopathology showed sheets of immature granulocytic cells. Immunohistochemical staining was positive for markers for myeloperoxidase, which supported the diagnosis of GS. CONCLUSIONS. Reports of a GS complicating the course of a MDS are few. Sites of detection of the extramedullary tumors (EMT) in MDS vary, although cutaneous sites predominate. An orbital site is a very rare localization of this tumor in adults. The case we report is the first one with an orbital granulocytic sarcoma in an MDS-type RAEB in an elderly patient.
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PMID:Orbital granulocytic sarcoma in a myelodysplastic syndrome. 1204 74

Granulocytic sarcoma is a rare extramedullary malignant mass composed of primitive cells of the granulocytic lineage. It can arise from any part of the body and is frequently associated with haematological diseases, commonly acute myeloid leukaemia. Rarely, it has been found in conjunction with myelodysplastic syndrome. We report a case of cutaneous granulocytic sarcoma in a 73-year-old lady. The patient presented with a two-month history of multiple skin nodules which were confirmed by skin biopsy to be granulocytic sarcoma. Bone marrow examination was consistent with myelodysplastic syndrome. Localised radiotherapy to the skin lesions were given. She died from septicaemia six months after presentation. The management of this condition presents a diagnostic and therapeutic dilemma for both the pathologist and physician. In cases which are poorly differentiated as in this case, histological diagnosis is particularly difficult. Its definitive diagnosis would then require the additional use of a broad panel of immunohistochemical and cytochemical stains.
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PMID:A case of generalised cutaneous granulocytic sarcoma in an elderly patient with myelodysplastic syndrome. 1258 8

A case of granulocytic sarcoma (chloroma) of hepatic localization is presented. It is a extramedullary strange tumour, composed of immature precursors of myeloid cells. Clinically it can show, before, during or after a acute myeloid leukemia, chronic myeloproliferative disorders or myelodysplastic syndromes. Our patient, 81 year-old male, presented a process of important acute jaundice, with negative image technics, what indicated us the intrahepatic origin, negative tumorals markers, negative serology and hepatic biopsy (the piece of greenish coloration is described) what showed a hepatic sinusoides diffuse infiltration by indifferentiation cellularity, with study immuno-histochemical that was positive for the myeloperoxydase, giving a diagnose compatible with hepatic infiltration for acute myeloid leukemia. The patient doesn't present affectation of peripheral blood, and he died for acute hepatic and renal failure after 8 days of entrance.
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PMID:[Hepatic granulocytic sarcoma: an unusual presentation]. 1275

Granulocytic sarcoma (extramedullary myelosarcoma, chloroma) is a rare extramedullary myeloid tumor which can occur at any anatomical site as isolated finding or associated with acute myelogenous leukemia (AML) or myelodysplastic syndrome (MDS). In this case, we describe a 71-year-old man who presented with incomplete paresis of the left arm, periorbital swelling, a maculopapular exanthema and organ involvement including testis and stomach. The tumors responded to combination chemotherapy and the patient fully recovered. However, after five months the patient relapsed and died quickly. This case confirms the importance of including granulocytic sarcoma in the differential diagnoses of a variety of diseases. In AML, the presence of granulocytic sarcoma is associated with worse overall survival. When diagnosed, it should be treated with intensive chemotherapy as soon as possible.
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PMID:Extramedullary acute myeloid leukemia (granulocytic sarcoma) with arm paresis, maculopapular exanthema and organ involvement. 1456 68

Extramedullary accumulation of myeloblasts or immature myeloid cells form tumors called myeloid sarcoma in the WHO classification. Such tumors develop in lymphoid organs, bone (skull, orbit, etc.), skin, soft tissue, various mucosae and organs, and the CNS. They may precede or occur concurrently with acute myeloid leukemia, or reveal blastic transformation of chronic myeloproliferative disorders or myelodysplastic syndromes. They may also reveal relapses in treated patients. They are constituted by a diffuse infiltrate made up of medium-to-large cells. The cells are difficult to identify. Imprints are very useful. Immunohistochemistry can help diagnose and distinguish four variants: granulocytic myeloperoxidase (MPO+, CD 68+ [KP1+/-, PGM1-] lysozyme+, CD 34+/-), monoblastic (MPO-, CD 68+, [KP1+, PGM1+] lysozyme+, CD 34-), myelomonoblastic (MPO-, CD 68+, [KP1+, PGM1+] lysozyme+, CD 34-), or megakaryoblastic (positivity for factor VIII, CD 61, CD 31). Immunohistochemistry sometimes demonstrates expression of CD 43, CD 7, CD 79a, and CD 56 (particularly the monoblastic variant with t[8;21]). Recently the demonstration of CD 99 and CD 117, which can now be done on paraffin sections, may be useful to identify blasts of granulocytic origin. The diagnosis is missed in about 50% of cases when immunohistochemistry is not used. Patients with myeloid sarcomas should be treated in the same way as patients with acute myeloblastic leukemia. Disease progression and prognosis are similar for the two conditions.
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PMID:Myeloid sarcoma: clinical and morphologic criteria useful for diagnosis. 1461 22

Granulocytic sarcoma is a rare orbital complication of acute leukemia. It concerns primarily children under 10 years of age suffering from primitive acute myeloid leukemia. As this type of symptom can sometimes affect the elderly during the acute phase of myelodysplastic syndrome, it raises a problem with diagnosis and consequently with therapeutic treatments. We report the case of a 77-year-old female patient who had acute inflammatory proptosis during regressive right ethmoiditis. The diagnosis was reached by computed tomography showing an extraconical intraorbital tumor sprouting from the sinus, and by clinical examination and confirmed by the hematological investigation. Acute proptosis developing in an acute myeloblastic leukemia context heavily swayed diagnosis towards orbital granulocytic sarcoma. Chemotherapy by cytarabine and hydroxycarbamide associated with intravenous corticoid therapy resulted in complete disappearance of the proptosis within 10 days. This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukemia. We also discuss different diagnostic methods and various therapeutic approaches, and analyze the disease course and patient follow-up.
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PMID:[Orbital granulocytic sarcoma revealing acute myeloblastic syndrome: a case study]. 1502 50

Granulocytic sarcomas are extramedullary tumors (EMD) of malignant myeloid precursor cells. EMD or granulocytic sarcoma of ovary is rare disease. A 15-year-old girl had complaints of abdominal pain and weight loss for 3 months. On physical examination, there were hepatosplenomegaly and a painless mass under the umbilicus. Breast development was grade II. There was no clitoris hypertrophy. Her labia majora were separate and vagina hypoplastic. Hemoglobin level was 9.3 g/dl, white blood cells count 2.8 x 10(6)/1, platelet count 31.6 x 10(9)/1. There were dysplastic features in the blood and bone marrow cells. There were 10 and 22% blasts in the peripheral blood smear and bone marrow, respectively. The levels of serum follicle stimulating and luteinizing hormones were high. An inguinal mass (diameter 9.5 x 7.6) cm was detected on computed tomography. The histopathological diagnosis of this was obtained from laporascopy was composed of ovotestis and there was marked blastic infiltration in this ovotestis which had myeloid markers on flow cytometry. In the immunohistochemical analyses of ovotestis and bone marrow, blasts were positive for LCA, CD-13, CD-33 and CD 68. The cytogenetic analysis of the bone marrow shaved 46 XY karyotype. No response was achieved with combination chemotherapy and the patient died from progressive leukemia. Here we report a rare patient with myelodysplastic syndrome, EMD and hermaphroditism. To our knowledge this is the first case of MDS, EMD and hermaphroditism.
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PMID:Granulocytic sarcoma of the ovotestis: an association of myelodysplastic syndrome and hermaphroditism. 1536 14


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