Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied ten cases of Japanese T-cell prolymphocytic leukemia (T-PLL) collected over the last 9 years. Median age was 61 years with a male predominance (M:F, 8:2). The main disease features were splenomegaly, lymphadenopathy, hepatomegaly, skin lesions and serous effusions. The clinical course was progressive with a median survival of 10 months. Immunophenotyping showed that the prolymphocytes had a post-thymic phenotype (TdT-, CD1a-, CD2+, CD3+, CD5+, CD7+) with a predominant CD4+ immunophenotype. Cytogenetic analysis showed no consistent abnormalities. 14q abnormality and trisomy 8q, which are frequently seen in T-PLL of Western countries, were found in only two and zero cases, respectively. We conclude that the clinical and biological characteristics of T-PLL in Japan are almost the same as those in Western countries. However, the cytogenetic findings of T-PLL in Japan might be different.
 ...
PMID:14q11 abnormality and trisomy 8q are not common in Japanese T-cell prolymphocytic leukemia. 984 13

The diagnosis of T-cell lymphomas by fine-needle aspiration biopsies (FNAB) is extremely difficult. This is mainly due to the rarity of the disease, the morphologic similarity to reactive lymphadenopathy, and the difficulty in identifying abnormal T-cell antigen expression. We studied FNAB of histologically proven T-cell lymphomas in an attempt to identify the salient cytomorphologic features as well as the surface marker attributes of the disease. Twenty cases were reviewed. The smears were evaluated for overall cytologic pattern and percentage of abnormal cells. A critical review of flow cytometric (FCM) antigen expression of the lymphomas was also performed. There were 6 female and 14 male patients, with an age range of 9-84 yr (median, 36 yr). Fourteen cases (70%) showed polymorphous smears, and 6 cases (30%) showed monomorphous smears. Abnormal cells ranged from 10-100% (median, 60%). Abnormal T-cell antigen expression by FCM analysis was seen in 17 cases (85%). The most common aberrant T-cell antigen pattern was loss of 3 or more pan-T-cell antigens (n = 10). The most common individual T-cell antigen loss was that of CD7 (n = 10), followed by loss of CD5 (n = 5). There was also loss of CD4 and CD8 (n = 5), loss of CD5 and CD7 (n = 5), complete loss of CD3 (n = 4), coexpression of CD4 and CD8 (n = 1), and partial loss of CD3 (n = 1). CD56 was expressed in 2 cases. CD1a was tested in one case and was positive. CD4/CD8 ratio was elevated (>2.5) in 9 cases (53%), with a range of 3/1-57/1 (median, 12/1). TCR gene rearrangement using PCR was positive in 7 of 9 tested cases. Our findings suggest that the diagnosis of peripheral T-cell lymphomas can be achieved by FNAB in the majority of cases through close analysis of the morphology. This can be supported by a critical analysis of the phenotype using two or three-color flow cytometry with an attempt at identification of one or more abnormal T-cell antigen expression and/or loss. This can be supplemented by CD4/CD8 ratios and T-cell receptor gene rearrangement analysis.
 ...
PMID:Diagnosis of peripheral T-cell lymphoma by fine-needle aspiration biopsy: a cytomorphologic and immunophenotypic approach. 1107 40

Sinus histocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease (RDD), is an idiopathic histiocytic proliferation affecting lymph nodes. Although extranodal involvement has been reported in diverse sites, central nervous system (CNS) manifestation, particularly in the absence of nodal disease is uncommon. We report 11 cases of RDD primary to the CNS without evidence of other sites of involvement. The cases included 7 males and 4 females ranging in age from 22 to 63 years (mean: 41 y). The patients presented with headaches, seizures, numbness, or paraplegia. Eight cases involved the cranial cavity and three cases, the spinal canal. Lesions were most often extra-axial and dura based. Only one presented in the CNS parenchyma. Histologically, the lesions consisted of variable numbers of pale-staining histocytes with emperipolesis often overshadowed by extensive lymphoplasmacytic infiltrates and fibrosis in the background. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein and CD68 and negative for CD1a. Treatment consisted of surgical biopsy or excision. Follow-up, available for 10 cases with intervals ranging from 5 days to 42 months (mean: 15 mo), disclosed one patient dying of operative complications 5 days after biopsy and nine patients with no evidence of disease progression RDD should be considered in the differential diagnosis of inflammatory lesions of the CNS. Our study suggests that this entity may have been misdiagnosed in the past as plasma cell granuloma or inflammatory pseudotumor.
 ...
PMID:Rosai-Dorfman disease isolated to the central nervous system: a report of 11 cases. 1126 22

Although there is a close association between Langerhans cell histiocytosis and malignant neoplasms, simultaneous occurrence of lymphoblastic lymphoma and Langerhans cell histiocytosis in the same lymph node is an extremely rare finding. Herein, we describe such a case in a 26-year-old woman who presented with progressive cervical lymphadenopathy. The lymphoma cells have an immature T-cell phenotype (terminal deoxynucleotidyl transferase(+), HLA-DR(+), CD34(+), CD38(+), and CD7(+)) with expression of both CD3 and CD79a on immunohistochemical stain. The Langerhans cells are present focally with the characteristic morphologic features and immunophenotype (CD1a(+) and S100(+)). The significance of CD79a coexpression in T-cell lymphoblastic lymphoma and the association between lymphoblastic lymphoma and Langerhans cell histiocytosis are discussed.
 ...
PMID:CD79a(+) T-cell lymphoblastic lymphoma with coexisting Langerhans cell histiocytosis. 1141 87

We analyzed the clinicopathologic and molecular findings in 26 adults (age 16-72 years) with T-cell acute lymphoblastic leukemia (T-ALL) and observed features that correlated with age. Patients older than 60 years (n = 5) had a low frequency of hepatosplenomegaly (0 [0%]), anterior mediastinal mass (1 [20%]), and lymphadenopathy (2 [40%]), and completely responded to chemotherapy (4 of 4). The T-ALL in this group commonly expressed myeloid antigens (4 [80%]), had lineage-inappropriate gene rearrangements (2/3 [67%]) and chromosome 2 deletion (3/4 [75%]), and exclusively used the V(III) or V(IV) families of the T-cell receptor (TCR) gamma gene. In comparison, patients 16 to 60 years old (n = 21) more commonly had an anterior mediastinal mass (8 [38%]), hepatosplenomegaly (10 [48%]), and lymphadenopathy (16 [76%]). The tumors in these patients commonly used the TCR gamma gene VI or V(II) families (17/25 total rearrangements [68%]). Myeloid antigen expression (5 [24%]) and lineage inappropriate gene rearrangements (4/15 [27%]) were uncommon. Within this group, CD1a expression correlated with age 28 to 60 years. These results illustrate considerable age-related heterogeneity in adult T-ALL, which may reflect differences in tumor cell maturation.
 ...
PMID:Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets. 1186 21

The cutaneous variant of Rosai-Dorfman disease is a rare benign lymphoproliferative disorder that occurs without lymphadenopathy or internal organ involvement. We present a 55-year-old woman with a diffuse exanthematous eruption that evolved into pruritic papulopetechial and purpuric lesions on the back and lower extremities, diagnosed clinically as a vasculitis. The patient's lesions cleared spontaneously with no recurrence. Histopathologic examination showed a dense dermal nodular lymphohistiocytic infiltrate with occasional eosinophils, plasma cells, and neutrophils and with numerous extravasated red blood cells. The large, foamy histiocytes exhibited abundant pale cytoplasm, feathery cytoplasmic borders, and emperipolesis. A few histiocytes had longitudinal nuclear grooves resembling Langerhans cells; however, these cells were positive for S-100 protein and negative for CD1a. The clinical presentation of the cutaneous variant of Rosai-Dorfman disease is generally nonspecific. We present this case because of the unusual clinical presentation mimicking a vasculitis.
 ...
PMID:Cutaneous sinus histiocytosis (Rosai-Dorfman disease) presenting clinically as vasculitis. 1200 23

Interdigitating dendritic cell sarcoma (IDCS) is an aggressive neoplasm of which fewer than 25 cases have been reported in the world literature. This malignancy is difficult to diagnose because of its rarity, and because of the subtle histopathologic features that distinguish IDCS from similar tumors arising from reticular cells. To date, there exists no consensus on a standard chemotherapeutic regimen for IDCS. Patients with this malignancy have been treated with chemotherapy regimens used against non-Hodgkin's lymphomas. Responses to these regimens have been variable, but mostly unsuccessful. In this article we describe a case of IDCS occurring in a 44 year old female who presented with abdominal pain and inguinal adenopathy. Staging of the tumor with CT scan, PET scan, and bone marrow biopsy demonstrated inguinal and abdominal lymphadenopathies, a large mass encasing the small bowel, and extensive liver infiltration. Morphologic and cytochemical analysis of biopsies from the abdominal mass and inguinal node were consistent with a diagnosis of IDCS, and immunohistochemical stains of the lymph node were positive for CLA, Kp-1, S-100, while negative for CD1a, CD3, CD20, CKER, and HMB45. Treatment of this patient with ABVD chemotherapy resulted in rapid clinical improvement with a marked decrease in tumor burden after two cycles of ABVD, and a complete response after six cycles of therapy.
 ...
PMID:Interdigitating dendritic cell sarcoma: a rare malignancy responsive to ABVD chemotherapy. 1215 70

Hematopoietic neoplasm coexpressing CD4 and CD56 includes a subset of acute myeloid leukemia with myelomonocytic differentiation, plasmacytoid monocyte tumor, and other immature hematopoietic neoplasms of undefined origin. Herein, we report a CD4+CD56+CD68+ hematopoietic tumor that was thought to be a tumor of plasmacytoid monocytes. This case is unique in the absence of accompanying myelomonocytic leukemia and the faint expression of cCD3 on the tumor cells. The patient was a 22-yr old man presented with multiple lymphadenopathy and an involvement of the bone marrow. Tumor cells were large and monomorphic with an angulated eosinophilic cytoplasm of moderate amount. Nuclei of most tumor cells were eccentric and round with one or two prominent nucleoli. Rough endoplasmic reticulum was prominent in electron microscopic examination. Tumor cells expressed CD4, CD7, CD10, CD45RB, CD56, CD68, and HLA-DR and were negative for CD1a, CD2, sCD3, CD5, CD13, CD14, CD20, CD33, CD34, CD43, CD45RA, TIA-1, S-100, and TdT. cCD3 was not detected in the immunostaining using paraffin tissue, but was faintly expressed in flow cytometry and immunostaining using a touch imprint slide. T-cell receptor gene rearrangement analysis and EBV in situ hybridization showed negative results. Cytochemically, myeloperoxidase, Sudan black B, and alpha naphthyl butyrate esterase were all negative.
 ...
PMID:CD4+CD56+CD68+hematopoietic tumor of probable plasmacytoid monocyte derivation with weak expression of cytoplasmic CD3. 1248 12

Rosai-Dorfman disease (RDD), previously known as sinus histiocytosis with massive lymphadenopathy, is a benign, idiopathic histiocytic proliferative disorder. It commonly affects lymph nodes, but any organ of the body may be involved. Histological findings include characteristic large, pale, histiocytic cells (Rosai-Dorfman cells) exhibiting cytophagocytosis. Immunohistochemically, these histiocytes are positive for S-100 protein and CD68, but stain negatively for CD1a. On electron microscopy, Birbeck granules are absent. RDD limited to the skin is rare, less than 30 cases having been reported to date. We present five further cases of purely cutaneous RDD. Three presented as solitary nodules and one as a large, well-circumscribed plaque. The fifth patient, who was HIV positive, had a rosacea-like facial eruption.
 ...
PMID:Five cases of cutaneous Rosai-Dorfman disease. 1255 21

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.
 ...
PMID:Congenital Rosai-Dorfman disease without lymphadenopathy. 1469 91


<< Previous 1 2 3 4 5 6 7 8 Next >>