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A J Norton

Publications and source records attributed to A J Norton.

At least 19 recordsLinked to original sources
PubMed2006

Monoclonal antibodies in the diagnosis of lymphoproliferative diseases of the orbit and orbital adnexae.

AIMS: To describe the value of monoclonal antibodies in the differential diagnosis of orbital lymphoproliferative disorders. METHODS: A total of 200 sequential cases of malignant lymphoma diagnosed at St Bartholomew's Hospital as part of the ocular lymphoma service at Moorfields Eye Hospital and the Institute of Ophthalmology have been examined. Cases were acquired between January 1998 and June 2005. Each case had detailed immunophenotypic analysis using a panel of monoclonal antibodies and was classified using the WHO classification of lymphoma. These cases are discussed in relation to earlier studies of orbital lymphoma reported by our group. RESULTS: Lymphomas fell into three main categories. Extranodal marginal zone lymphoma was the largest group with 151 cases, arising within the orbital soft tissue, conjunctiva and lacrimal gland. Cases arising in the conjunctiva and lacrimal gland showed a higher female predominance than those arising within the deeper soft tissue. A small number of cases were associated with organ specific autoimmunity, including thyroid eye disease complicating Graves' disease. Follicular lymphoma and diffuse large B-cell lymphoma formed the next two groups, occurring with equal frequency. Many of the follicular lymphomas had evidence of disseminated disease on completion of staging. A miscellaneous group of T-cell and B-cell lymphomas formed a minority of cases during the study period. CONCLUSION: Extranodal marginal zone lymphoma is the most frequent type of primary orbital and orbital adnexal lymphoma. Its major differential diagnosis is with orbital lymphoid hyperplasia, chronic dacryoadenitis, and follicular conjunctivitis. Systemic types of lymphoma may present within the orbit or involve the orbit secondarily.

Antibodies, Monoclonal↗
PubMed2005

A limited role for TP53 mutation in the transformation of follicular lymphoma to diffuse large B-cell lymphoma.

The role of TP53 mutation in transformation of follicular lymphoma (FL) to diffuse large B-cell lymphoma (t-FL) was examined in a panel of 91 lymph node biopsies derived from 29 patients pre- and post-transformation. The entire TP53 coding sequence was screened and immunocytochemistry performed to determine expression of p53 and its key regulator MDM2. A total of 10 mutations were detected in eight patients (28%), although none were present at FL diagnosis. Mutations were not detected solely at the time of transformation; in three patients, mutated TP53 arose in at least one antecedent FL sample (6 months, 2.5 years and 4 years prior to transformation). Loss of heterozygosity at the TP53 locus occurred in 2/20 informative patients (only in t-FL samples). p53 staining was positive in 82% (9/11) of available biopsies with a missense mutation, and negative in 71% (45/63) with wtTP53. MDM2 expression was significantly higher in t-FL samples (mean 72% positive; 95% confidence interval (95% CI) 68-76%) than FL (mean 58% positive; 95% CI 54-62%) (P<0.001) but did not correlate with TP53 status. TP53 mutation has only a limited role in the transformation of FL, exerting a heterogeneous influence upon phenotypic change. In contrast, dysregulation of MDM2 is frequent and may provide a more rational therapeutic target..

Cell Transformation, Neoplastic↗
PubMed2004

Tositumomab and iodine I 131 tositumomab for recurrent indolent and transformed B-cell non-Hodgkin's lymphoma.

PURPOSE: An open-label phase II study was conducted at two centers to establish the efficacy and safety of tositumomab and iodine I 131 tositumomab at first or second recurrence of indolent or transformed indolent B-cell lymphoma. PATIENTS AND METHODS: A single dosimetric dose was followed at 7 to 14 days by the patient-specific administered radioactivity required to deliver a total body dose of 0.75 Gy (reduced to 0.65 Gy for patients with platelets counts of 100 to 149 x 10(9)/L). Forty of 41 patients received both infusions. RESULTS: Thirty-one of 41 patients (76%) responded, with 20 patients (49%) achieving either a complete (CR) or unconfirmed complete remission [CR(u)] and 11 patients (27%) achieving a partial remission. Response rates were similar in both indolent (76%) and transformed disease (71%). The overall median duration of remission was 1.3 years. The median duration of remission has not yet been reached for those patients who achieved a CR or CR(u). Eleven patients continue in CR or CR(u) between 2.6+ and 5.2+ years after therapy. Therapy was well tolerated; hematologic toxicity was the principal adverse event. Grade 3 or 4 anemia, neutropenia, and thrombocytopenia were observed in 5%, 45%, and 32% of patients, respectively. Secondary myelodysplasia has occurred in one patient. Four patients developed human antimouse antibodies after therapy. Five of 38 assessable patients have developed an elevated thyroid-stimulating hormone; treatment with thyroxine has been initiated in one patient. CONCLUSION: High overall and CR rates were observed after a single dose of tositumomab and iodine I 131 tositumomab in this patient group. Toxicity was modest and easily managed.

Adult↗
PubMed2004

CD23 expression in mediastinal large B-cell lymphomas.

AIMS: Mediastinal large B-cell lymphoma (MLBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL) in the WHO classification with peculiar features, such as female prevalence, young patient age and bulky presentation. It shows a B-cell phenotype with variable expression of surface immunoglobulin, negative CD21 and CD10 and positive CD30 in a large number of cases. An origin from activated thymic B cells has been suggested in several studies. A subpopulation of large, dendritic cells (asteroid cells) strongly expressing CD23 has been identified amongst thymic B cells and these could represent the normal cellular counterpart for this type of primary mediastinal large cell lymphoma. METHODS AND RESULTS: To explore this possibility, we immunostained 24 cases of primary mediastinal lymphomas and 100 cases of non-mediastinal, nodal and extranodal, DLBCLs for CD23 in routinely processed paraffin-embedded tissues. CONCLUSIONS: Our results show that a vast majority (70%) of mediastinal lymphomas strongly express CD23 whilst the same antigen is expressed in only 15% of non-mediastinal nodal DLBCLs and 9% of non-mediastinal extranodal DLBCLs. These results support the hypothesis that most cases of MLBCL arise from activated dendritic thymic B cells. We also suggest that CD23 should be included in the panel of antibodies currently used to characterize this subtype of DLBCL.

Adolescent↗
PubMed2001

Mitoxantrone is superior to doxorubicin in a multiagent weekly regimen for patients older than 60 with high-grade lymphoma: results of a BNLI randomized trial of PAdriaCEBO versus PMitCEBO.

A prospective, multicenter, randomized trial was undertaken to compare the efficacy and toxicity of adriamycin with mitoxantrone within a 6-drug combination chemotherapy regimen for elderly patients (older than 60 years) with high-grade non-Hodgkin lymphoma (HGL) given for a minimum of 8 weeks. A total of 516 previously untreated patients aged older than 60 years were randomized to receive 1 of 2 anthracycline-containing regimens: adriamycin, 35 mg/m(2) intravenously (IV) on day 1 (n = 259), or mitoxantrone, 7 mg/m(2) IV on day 1 (n = 257); with prednisolone, 50 mg orally on days 1 to 14; cyclophosphamide, 300 mg/m(2) IV on day 1; etoposide, 150 mg/m(2) IV on day 1; vincristine, 1.4 mg/m(2) IV on day 8; and bleomycin, 10 mg/m(2) IV on day 8. Each 2-week cycle was administered for a minimum of 8 weeks in the absence of progression. Forty-three patients were ineligible for analysis. The overall and complete remission rates were 78% and 60% for patients receiving PMitCEBO and 69% and 52% for patients receiving PAdriaCEBO (P =.05, P =.12, respectively). Overall survival was significantly better with PMitCEBO than PAdriaCEBO (P =.0067). However, relapse-free survival was not significantly different (P =.16). At 4 years, 28% of PAdriaCEBO patients and 50% of PMitCEBO patients were alive (P =.0001). Ann Arbor stage III/IV, World Health Organization performance status 2-4, and elevated lactate dehydrogenase negatively influenced overall survival from diagnosis. In conclusion, the PMitCEBO 8-week combination chemotherapy regimen offers high response rates, durable remissions, and acceptable toxicity in elderly patients with HGL.

Aged↗
PubMed2001

High-dose therapy and autologous stem-cell support for chemosensitive transformed low-grade follicular non-Hodgkin's lymphoma: a case-matched study from the European Bone Marrow Transplant Registry.

PURPOSE: To assess the outcome of high-dose therapy with autologous stem-cell support in patients with histologic transformation of low-grade follicular non-Hodgkin's lymphoma (NHL) and identify significant prognostic factors, as well as to compare survival of these patients with that of patients with matched low-grade and de novo high- or intermediate-grade NHL undergoing the same procedure. PATIENTS AND METHODS: Fifty patients with transformed low-grade NHL have been reported to the European Bone Marrow Transplant registry. Outcome from high-dose therapy and significant prognostic factors were analyzed. Their survival was also compared with that of 200 patients with matched low-grade NHL and 200 patients with matched de novo high- or intermediate-grade NHL by a case-matched analysis. RESULTS: The procedure-related death rate among the 50 transformed NHL patients was 18%. Overall survival (OS) and progression-free survival (PFS) rates were 51% and 30% at 5 years, respectively. Median PFS time was 13 months. Raised lactate dehydrogenase levels at transformation (P =.0031) was identified as the only adverse significant predictor of PFS on multivariate analysis. A subgroup of patients with residual chemosensitive disease who attained complete remission after high-dose therapy had the best outcome, with an OS at 5 years of 69%. A comparison with matched patients with low-grade disease and with de novo high- or intermediate-grade lymphoma showed no significant difference in OS (P =.939 and P =.438, respectively). CONCLUSION: Patients with chemosensitive transformed lymphoma should be seriously considered for high-dose therapy and autologous stem-cell support.

Adult↗
PubMed2001

Loss of CD20 expression following treatment with rituximab (chimaeric monoclonal anti-CD20): a retrospective cohort analysis.

A retrospective analysis of CD20 expression following rituximab for B-cell non-Hodgkin's lymphoma demonstrated a significant change in immunophenotype in 6/25 (24%) patients with persistent bone marrow (BM) infiltration. In three out of six patients, the B cells were uniformly CD20-/CD79alpha+, consistent with frank loss of CD20 expression. In the remaining three cases, the BM infiltrate was predominantly (> 80%) CD20-/CD79alpha+. Two of the former but none of the latter three cases achieved a clinical response. In three further cases, the post-treatment BM infiltrate was composed entirely of benign or reactive CD3+ T cells. Frank loss of CD20 was not seen in 25 post-treatment lymph node biopsies. Immunophenotyping is therefore an important adjunct in the diagnosis of BM infiltration following rituximab.

Antibodies, Monoclonal↗
PubMed2001

Imaging-guided core biopsy for the diagnosis of malignant tumors in pediatric patients.

OBJECTIVE: We evaluated the ability of imaging-guided core biopsy to obtain sufficient tissue from pediatric tumors for a definitive diagnosis of malignancy on which treatment could be based. MATERIALS AND METHODS: Thirty-four biopsies (biopsies of the abdomen, 32; of the chest, 2) were performed on 34 children at presentation under CT or sonographic guidance using 14-, 18-, or both 14- and 18-gauge needles. A minimum of two tissue cores was obtained. Most biopsies were performed under general anesthesia, permitting other procedures to be performed. The biopsy results were confirmed by subsequent surgical pathology, bone marrow biopsy, biochemical or clinical features, and follow-up examination. RESULTS: The needle biopsy diagnoses were nephroblastoma (n = 11), neuroblastoma (n = 7), renal cell carcinoma (n = 2), synovial sarcoma (n = 1), non-Hodgkin's lymphoma (n = 2), clear cell sarcoma (n = 1), rhabdoid tumor (n = 1), pulmonary blastoma (n = 2), embryonal rhabdomyosarcoma (n 1), germ cell tumor (n = 1), adrenal carcinoma (n = 1), inflammatory tissue (n = 2), desmoplastic tumor of the mesentery (n = 1), and primitive neuroectodermal tumor (n = 1). In 28 patients, the results were confirmed as correct (22 with surgery and 6 with follow-up examination). Four patients required additional biopsy. In two of these patients, the core biopsy showed inflammatory tissue only, and an open biopsy of a different site was performed; the other two patients did not respond to therapy on the basis of needle biopsy results, and an open biopsy altered the diagnosis. Two patients with widespread disease were excluded because they did not respond to treatment and were too ill to undergo an open biopsy. Only one significant complication was recorded. CONCLUSION: Imaging-guided core biopsy is a safe and reliable means of obtaining sufficient tissue to make a confident histologic diagnosis of malignant pediatric tumors in a high percentage of patients.

Abdominal Neoplasms↗
PubMed2000

Malignant lymphoma of the urinary bladder: a clinicopathological study of 11 cases.

AIM: To report the clinical and histological features and outcome of primary and secondary malignant lymphomas of the urinary bladder. METHODS: Eleven cases of malignant lymphoma of the urinary bladder were obtained from the registry of cases at St Bartholomews and the Royal London Hospitals. The lymphomas were classified on the basis of their morphology and immunophenotype, and the clinical records were reviewed. RESULTS: There were six primary lymphomas: three extranodal marginal zone lymphomas of mucosa associated lymphoid tissue (MALT) type and three diffuse large B cell lymphomas. Of the five secondary cases, four were diffuse large B cell lymphomas, one secondary to a systemic follicular follicle centre lymphoma, and one nodular sclerosis Hodgkins disease. Four patients with secondary lymphoma for whom follow up was available had died of disease within 13 months of diagnosis. Primary lymphomas followed a more indolent course. In one case, there was evidence of transformation from low grade MALT-type to diffuse large B cell lymphoma. The most common presenting symptom was haematuria. Cystoscopic appearances were of solid, sometimes necrotic tumours resembling transitional cell carcinoma, and in one case the tumours were multiple. These cases represented 0.2% of all bladder neoplasms. CONCLUSIONS: Diffuse large B cell lymphoma and MALT-type lymphoma are the most common primary malignant lymphomas of the bladder. Lymphoepithelial lesions in MALT-type lymphoma involve transitional epithelium, and their presence in high grade lymphoma suggests a primary origin owing to transformation of low grade MALT-type lymphoma. Primary and secondary diffuse large B cell lymphomas of the bladder are histologically similar, but the prognosis of the former is favourable.

Adult↗
PubMed2000

High-dose therapy with autologous bone marrow support as consolidation of remission in follicular lymphoma: long-term clinical and molecular follow-up.

PURPOSE: To evaluate the long-term results of high-dose therapy (HDT) in follicular lymphoma, with specific emphasis on the prognostic significance of polymerase chain reaction (PCR)-detectable Bcl-2/IgH rearrangements. PATIENTS AND METHODS: Between June 1985 and October 1995, 99 patients with follicular lymphoma received HDT as consolidation of second or subsequent remission. Bone marrow was treated in vitro with anti-B-cell antibodies and complement. RESULTS: Sixty-five patients remained alive, 49 treatment-failure free, with a median follow-up of 5.5 years (range, 1.5 to 12.5 years). Four "early" and 10 "late" deaths occurred from treatment-related causes; seven of the latter were due to secondary myelodysplasia (s-MDS) or secondary acute myeloblastic leukemia. Overall, 12 (12%) of the 99 patients developed s-MDS or acute myeloblastic leukemia. Kaplan-Meier estimates of freedom from recurrence (FFR) and survival rates at 5 years were 63% (95% confidence interval [CI], 52% to 72%) and 69% (95% CI, 58% to 78%), respectively. For all 99 patients, in multivariate analysis, absence of the Bcl-2/IgH rearrangement at the time of diagnosis (hazards ratio [HR], 0.39; P =.04) and three or fewer treatment episodes before HDT (HR, 0.03; P =.001) were significant prognostic factors for improved survival. For patients bearing Bcl-2/IgH rearrangements, in univariate and multivariate analyses, absence of a PCR-detectable Bcl-2/IgH rearrangement during follow-up was associated with a significantly lower risk of recurrence (adjusted HR, 0.13; P <.001) and death (HR, 0.25; P =.02), whereas the PCR status of the reinfused bone marrow did not correlate with outcome. CONCLUSION: Prolonged FFR can be achieved in patients with follicular lymphoma after HDT, but as yet there is no survival advantage compared with conventional treatment. These results confirm that elimination of cells bearing the Bcl-2/IgH rearrangement is highly desirable and should be attempted. The incidence of s-MDS is of increasing concern in this setting.

Adult↗
PubMed1999

Monoclonal Epstein-Barr virus-related lymphoproliferative disorder following adult acute lymphoblastic leukaemia.

A 31-year-old patient in remission of acute lymphoblastic leukaemia (ALL), receiving oral maintenance chemotherapy (6-mercaptopurine, methotrexate (MTX), cyclophosphamide), developed a monoclonal, Epstein-Barr virus (EBV)-related lymphoproliferative disorder (LPD). Treatment consisted of excisional biopsy and the discontinuation of maintenance chemotherapy. To our knowledge, this is the first such report in an adult. The histological similarity to previous reports of 'lymphomatoid granulomatosis' following paediatric ALL suggests that they are the same disease. MTX may play a central role in the development of LPD in this setting. Although it is a rare complication of ALL, EBV-related LPD should be considered in patients who develop lymphadenopathy.

Adult↗
PubMed1999

Classification of cutaneous lymphoma: a critical appraisal of recent proposals.

The classification of cutaneous lymphoma is a contentious issue. In this short review the merits of REAL classification and EORTC classification for cutaneous lymphoproliferative disease are examined. Points of terminological confusion between the two schemes are considered and a brief account of less common or ambiguous lymphoma types is provided.

Child↗
PubMed1999

Multicenter phase II study of fludarabine phosphate for patients with newly diagnosed lymphoplasmacytoid lymphoma, Waldenström's macroglobulinemia, and mantle-cell lymphoma.

PURPOSE: Fludarabine phosphate (F-AMP) has significant activity in follicular lymphoma and in B-cell chronic lymphatic leukemia, where it has demonstrated high complete response (CR) rates. Lymphoplasmacytoid (LPC) lymphoma, Waldenstrom's macroglobulinemia (WM), and mantle-cell lymphoma (MCL) also present with advanced-stage disease and are incurable with standard alkylator-based chemotherapy. A phase II trial was undertaken to determine the activity of F-AMP in patients newly diagnosed with these diseases. PATIENTS AND METHODS: Between 1992 and 1996, 78 patients (aged 18 to 75 years) received intravenous F-AMP (25 mg/m2/d for 5 days, every 4 weeks) until maximum response, plus two further cycles as consolidation. The primary end point was response rate; secondary end points included time to progression (TTP), duration of response, and overall survival (OS). RESULTS: Forty-four (62%) of 71 assessable patients had a response to F-AMP (LPC lymphoma, 63%; WM, 79%; MCL, 41%); the CR rate was 15%. At a median follow-up of 1.5 years, 19 of 44 responding patients have had progression of lymphoma; the median duration of response was 2.5 years. The median survival has not yet been reached. There was no significant difference in the duration of response or OS between patients with different histologies; TTP was shorter in patients with MCL (P = .015). Myelosuppression was relatively common, and the treatment-related mortality (TRM) was 5%, mostly associated with pancytopenia and infection. CONCLUSION: Single-agent fludarabine phosphate is active in previously untreated LPC lymphoma and WM, with only moderate activity in MCL. However, the CR rate is low, and the TRM is relatively high. Its role in combination chemotherapy remains to be demonstrated.

Adult↗
PubMed1999

Immunocytoma: a retrospective analysis from St Bartholomew's Hospital-1972 to 1996.

PURPOSE: To analyze the presentation features and outcome for patients with immunocytoma (IMC) managed at St Bartholomew's Hospital (SBH), London, United Kingdom, between 1972 and 1996. Outcome was compared with that of patients with small lymphocytic lymphoma (SLL)/B-cell chronic lymphocytic leukemia (B-CLL) treated at SBH during the same period. PATIENTS AND METHODS: One hundred twenty-six patients with newly diagnosed IMC were identified. Patients were subclassified (using the Kiel classification) as having lymphoplasmacytoid (n =92), lymphoplasmacytic (n = 24), polymorphous (n = 9), or undetermined (n = 1) IMC. Six patients (5%) had stage I to IIE disease; the rest had advanced disease. Treatment was given according to disease stage. Seven patients were managed expectantly. RESULTS: Eighty-two (69%) of 119 patients responded to treatment, but complete remission was seen in only 15 (13%) of 119. Treatment failed in 29 (24%) of 119 patients. There were three treatment-related deaths; five patients were not assessable for response. When survival of patients with IMC was compared with that of patients with B-CLL/SLL, a significant difference was found (P <. 01); this difference was maintained when only patients in whom the diagnosis was based on lymph node biopsy were considered (P =.01). A comparison of the three IMC subgroups showed that there was a trend (P =.06) toward a difference between B-CLL/SLL and the lymphoplasmacytoid subtype. CONCLUSION: Patients diagnosed with IMC are generally older and present with advanced disease. Conventional therapies usually result in incomplete responses of short duration. Overall, these results support the proposed World Health Organization reclassification of IMC to include lymphoplasmacytoid lymphoma (Kiel classification) as a variant of B-CLL/SLL.

Adult↗
PubMed1998

A survey of competencies and concerns in end-of-life care for physician trainees.

A survey was developed to explore physician trainee competencies and concerns surrounding end-of-life care. Thirty-one medical students, interns, and residents from the Department of Internal Medicine completed the survey in August 1996. The survey instrument found differing levels of competence/concern among medical students, interns, and residents. Self-reported competence increased with level of training. All trainees indicated the least comfort around discussions of hydration and feeding withdrawal. Both residents and interns indicated concern about potential illegality, breach of ethics or potential malpractice when reviewing eight currently legal and ethical end-of-life scenarios involving pain management or treatment withdrawal. Pain management, ethical issues, and delirium were the top three topics for which residents indicated an interest in future educational sessions. Results from the survey will be used to guide the development of educational initiatives that address trainee concerns. The competence/concern survey adds an important dimension to understanding how best to incorporate end-of-life education into residency training programs.

Clinical Competence↗
PubMed1998

High-dose therapy with autologous haematopoietic support in patients with transformed follicular lymphoma: a study of 27 patients from a single centre.

BACKGROUND: The prognosis of patients with transformed follicular lymphoma (FL-t) is poor. The use of high-dose therapy (HDT) with autologous haematopoietic support was therefore evaluated as consolidation of remission. PATIENTS AND METHODS: Twenty-seven patients received high-dose cyclophosphamide and total body irradiation (cyclo + TBI) with autologous bone marrow (BM; n = 24) or peripheral blood progenitor cell support (PBPC; n = 3). BM was treated in vitro with anti-B cell antibodies and complement. Nineteen of 27 patients were treated in first stable remission following transformation. Eight other patients with a history of transformation were treated following a subsequent recurrence of follicular lymphoma (FL). RESULTS: With a median follow-up of 2.4 years, 14 of 27 patients remain alive and in remission; five are alive and free of disease at more than four years. The median survival is 8.5 years. There were two 'early' treatment-related deaths of respiratory failure, and two 'late' deaths of myelodysplastic syndrome (MDS) in remission of lymphoma at 2.8 and 8.5 years. Seven of nine patients having had a recurrence underwent re-biopsy. In two, histology revealed FL, in five, transformed follicular lymphoma. One of the patients with recurrent FL is alive without further therapy, and two of five patients with recurrent FL-t are alive and in remission after further chemotherapy. CONCLUSIONS: It is appropriate to consider HDT for younger patients with FL-t in remission. Repeat biopsy should be considered for patients with recurrent disease. There is a risk of late MDS in patients undergoing this treatment.

Adult↗
PubMed1998

True histiocytic lymphoma: a morphologic, immunohistochemical, and molecular genetic study of 13 cases.

We describe the morphologic, immunohistologic, and genotypic characteristics of 13 cases of true histiocytic lymphomas. Six cases presented with primary gastrointestinal involvement, five with lymphadenopathy, the other sites involved being the bone marrow and the skin. The neoplastic cells displayed large abundant eosinophilic cytoplasm, occasionally vacuolated with folded or bizarre-shaped nuclei with prominent nucleoli. Mitotic figures were numerous. Multinucleated cells were common. The pattern of growth was usually diffuse and noncohesive. Spindle cell sarcoma-like areas were evident in five cases, with a prominent foam cell component in four cases. All cases expressed histiocyte-associated markers (CD68, lysozyme, alpha-1-antitrypsin), CD45 or CD45RO, and were negative for CD1a, epithelial, and B- and T-cell lineage-specific markers. Reactivity for S-100 was observed in a variable proportion of cells in 11 cases. The proliferation fraction varied from 3 to 88%. Genotypic analysis for T-cell receptor or immunoglobulin gene rearrangement demonstrated a germline configuration in all cases. We demonstrate that true histiocytic lymphoma is a rare distinctive pathologic entity that may be defined by immunohistochemical criteria and that recognition among histiocytic disorders is important for clinical and prognosis reasons.

Adult↗