Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0019829 (Hodgkin's disease)
 30,247 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A previously healthy 12-year-old boy presented with acute onset of proptosis of his left eye. CT scan demonstrated a mass involving the left orbit, left maxillary sinus, and left ethmoid sinus with extension through the cribriform plate into the anterior cranial fossa. Incisional biopsy of the mass revealed a precursor B-cell lymphoblastic lymphoma. Precursor B-cell lymphoblastic lymphoma is a rare type of non-Hodgkin's lymphoma seen exclusively in children and young adults. This is the first reported case of precursor B-cell lymphoblastic lymphoma presenting in the orbit. Treatment is primarily by systemic chemotherapy and is potentially curative. The principal role of the ophthalmologist is in diagnosis and monitoring of such patients. The clinical features and multidisciplinary diagnosis and management of childhood non-Hodgkin's lymphomas are reviewed.
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PMID:Precursor B-cell lymphoblastic lymphoma presenting as an orbital mass. 1204 94

DP-1 is a G1 cell cycle-related protein that forms heterodimers with E2F, a family of transcriptional factors regulating the expression of genes important for G1 to S progression. Although the exact role of DP-1 is not well understood, it has been shown to stabilize DNA binding of E2F proteins. By immunohistochemistry, the authors examined the expression of DP-1 in lymphoid tissues, including 8 cases of reactive follicular hyperplasia and 69 cases of B-cell non-Hodgkin lymphoma. The expression of the cell cycle-related proteins E2F-1 and Ki-67 was also assessed. Scoring was based on the proportion of labeled nuclei (1-10%, 11-25%, 26-50%, and > 50%). In reactive follicular hyperplasia, staining for DP-1, E2F-1, and Ki-67 was largely confined to the germinal centers. All 25 cases of follicular lymphoma, regardless of grade, had a high proportion (> 50%) of DP-1-positive cells but a lower proportion of cells marking for E2F-1 and Ki-67 (P < 0.001). The diffuse large B-cell lymphomas (n = 24) had high DP-1 and Ki-67 scores but low E2F-1 scores (P < 0.001). Small lymphocytic (n = 10), marginal zone (n = 3), and mantle cell lymphomas (n = 5) contained relatively low proportions of cells labeled for all three markers. Precursor B-cell lymphoblastic lymphoma (n = 2) displayed high proportions of cells positive for DP-1, Ki-67, and E2F-1 (> 50% in both cases). Except in follicular center cell lesions, DP-1 expression generally correlated with that of Ki-67. However, the expression of DP-1 was discordant with that of E2F-1 in benign and malignant follicular center cells, suggesting that DP-1 may have functions other than facilitating E2F-1-dependent gene regulation and cell cycle progression in these neoplasms.
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PMID:Immunohistochemical expression of the transcription factor DP-1 and its heterodimeric partner E2F-1 in non-Hodgkin lymphoma. 1260

Precursor B-cell lymphoblastic lymphoma (PBLL) is a rare subtype of childhood non-Hodgkin lymphoma (NHL). Most lymphoblastic lymphomas have a T-cell immunophenotype, but a small distinct proportion is of precursor B-cell origin. Skin and bone involvement is seen more commonly in this clinical variant. Primary cutaneous PBLL is rare. We describe an 8-year-old girl who presented with an asymptomatic nodule on the left upper arm. Histopathological features were consistent with pre-B-cell lymphoblastic lymphoma, and staging investigations excluded extracutaneous disease, resulting in a diagnosis of primary cutaneous PBLL. The child was started on induction chemotherapy, UKALL 2003 regimen B. She developed disseminated varicella zoster virus and died despite treatment. We discuss previously reported cases of primary cutaneous PBLL and their outcomes.
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PMID:Primary cutaneous precursor B cell lymphoblastic lymphoma in a child, complicated by fatal disseminated varicella zoster virus. 2595 84

Precursor B-cell lymphoblastic lymphoma, a type of non-Hodgkin lymphoma, is common in children. It is mostly extranodal; skin, bone and soft tissue are more often involved. However, isolated peritoneal presentation is rare. In this article a unique pediatric case of isolated omental precursor B-cell lymphobastic lymphoma is presented.
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PMID:A Rare Presentation of Precursor B-cell Lymphoblastic Lymphoma in a Child. 3041 Aug 44

Precursor B-cell lymphoblastic lymphoma (PBLL) is a rare subtype of non-Hodgkin lymphoma originating from B-cell precursors. PBLL, as a solitary mass lesion affecting the central nervous system without leukemic disease at presentation, is quite uncommon. Here we report a rare PBLL case with Philadelphia chromosome positivity. The 44-year-old male presented a solitary bulky mass primarily involving the left frontotemporal lobes and extended into the infratemporal fossa. Pretreatment PET/CT imaging showed avid 18F-fluorodeoxyglucose (18F-FDG) uptake of the lesion. By aggressive chemotherapy and imatinib maintenance treatment, the patient achieved and remained in complete remission on another two consecutive PET/CT imaging follow-ups.
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PMID:Successful Treatment for Adult Precursor B-Cell Lymphoblastic Lymphoma Involving Central Nervous System Monitored by 18F-Fluorodeoxyglucose PET/CT Imaging. 3226 34