Characterization of extramedullary tumors in a case of Ph-positive chronic myelogenous leukemia: possible involvement of immature T lymphocytes

A 42-year-old male with chronic myelogenous leukemia (CML) developed acute transformation associated with subcutaneous tumors. Histopathologic examinations of the tumors were done on two occasions; the first study revealed reticulum cell sarcoma-like features, and the second suggested a blastoma. Chromosomal analysis showed that the cells of the tumors originated from the CML clone. The cells had a negative reaction for myeloperoxidase by electron microscopy. Furthermore, biochemical and surface marker studies revealed that the tumor cells contained a significant terminal transferase activity. However, they did not express E- or EAC-rosette receptors, Ia-like antigens, or common ALL antigens.     

Chronic lymphocytic leukemia: the pathologist's view of lymph node microenvironment

Chronic lymphocytic leukemia (CLL), an indolent B-cell malignancy frequently diagnosed in the elderly, is characterized by the relentless accumulation of CD5+ monoclonal B cells that proliferate in the appropriate tissue microenvironments. Despite many advances achieved by molecular and functional studies, our knowledge of the reciprocal relationship between the CLL cell and its microenvironment at the tissue level is still largely incomplete. In this review we present the relevant current information on the tissue microenvironmental features of CLL, focusing on the events that appear to occur in the lymph node. Special attention is devoted to analyzing the properties of both neoplastic and nonneoplastic bystander cells within proliferation centers, the mysterious structures that likely represent the actual proliferative compartment.     

累及淋巴结的肾脏血管平滑肌脂肪瘤一例

患者男,33岁,乏力,低热1年,左侧腰部阵发性绞痛反复发作1个月于2002年4月5日入院,体检：无发热、发冷,夜间盗汗及尿频,尿急、尿痛、尿血等病史。B超,X线及CT检查均提示：左肾占位性病变,肾细胞癌可能性大。实验室检查无异常。专科检查：双肾区对称无隆起,左肾区叩击痛,左输尿管走行区轻度压痛,膀胱区隆起无压痛。临床诊断;左肾肿瘤。     

Hyperplastic mesothelial cells in mediastinal lymph node sinuses with extranodal lymphatic involvement

We describe a patient with hyperplastic mesothelial cells localized to mediastinal lymph node sinuses. These mesothelial cells were originally misdiagnosed as metastatic carcinoma, and the patient received radiotherapy. Histologic review, immunohistochemistry, and ultrastructural studies confirmed mesothelial cell origin. These nodal mesothelial cells were associated with pericardial and pleural effusions. Extranodal lymphatics also contained hyperplastic mesothelial cells, confirming their mode of lymphatic transport to node sinuses. This finding supports the theory that hyperplastic mesothelial cells derive from reactive serosal mesothelium and are dislodged into draining lymphatics. This is the first report, to our knowledge, that demonstrates the pathogenetic significance of this lymphatic transport mechanism. Awareness of intralymphatic and nodal benign hyperplastic mesothelial cells and their mimicry of invasive malignant neoplasms is important for accurate diagnoses and appropriate therapy.     

Angiomyolipoma of the kidney with lymph node involvement.

A case of renal angiomyolipoma with regional lymph node involvement is added to 20 cases previously reported. This represents the 11th case reported in a non-tuberous sclerosis patient. The electron microscopic features of the tumor are discussed and correlated with the light microscopic findings.     

Lymphoid blast crises of chronic myelogenous leukemia represent stages in the development of B-cell precursors

The origin and stage of differentiation of the blast-crisis cells in chronic myelogenous leukemia have remained uncertain. Because immunoglobulin heavy-chain and light-chain genes must undergo a DNA rearrangement during B-cell development but rarely do so in human non-B-cell lineages, we examined these genes in 18 episodes of chronic myelogenous leukemia. In eight of nine episodes of lymphoid blast crisis, heavy-chain genes were rearranged, and in three, rearrangements in light-chain genes were also present. In contrast, cells from chronic myeloid, myeloid blast, and erythroid-like phases retained germ-like immunoglobulin genes. The observed phenotypic markers and gene configurations revealed that most lymphoid blast crises represent stages of development of B-cell precursors. In two separate episodes of lymphoid crisis, cells from a single patient possessed identical heavy-chain but different light-chain-gene configurations. Thus, the precursor cells that monoclonally expand to produce a lymphoid crisis are capable of immunoglobulin-gene rearrangements and represent discrete steps in early B-cell maturation.     

Chronic myelogenous leukemia with reticulum-cell-sarcoma-like cell proliferation--significance of eosinophilic granules in sarcomatous cells.

A case of chronic myelogenous leukemia (CML) associated with proliferation of atypical cells resembling those of reticulum cell sarcoma (RCS-like cells) is presented. A number of immature eosinophils were present mingled with ordinary leukemic cells, which infiltrated in the bone marrow, lymph nodes, spleen, liver, lungs and testes. RCS-like cells either formed solitary nodular foci or randomly mingled with infiltrating leukemic cells. Charcot-Leyden crystals were seen in some areas where RCS-like cells proliferated. As a peculiar feature the presence of eosinophilic granules in some of the RCS-like cells was noted. They were proved to be immature form of specific granules of eosinophils by their staining properties and ultrastructural aspects. Based on these findings the myelogenous origin of RCS-like cells is suggested. The patient died of cerebral complication of aspergillosis.     

Breakpoint zone of bcr in chronic myelogenous leukemia does not correlate with disease phase or prognosis

The presence of a zone 4 rearrangement in the bcr of two chronic myelogenous leukemia (CML) cell lines and 25 CML patients was investigated by hybridization of HindIII-EcoRI double-digested DNA with the homologous HindIII-BglII breakpoint cluster region (bcr) probe. A rearrangement in zone 4 was found in the EM2 cell line and in 11 of the 25 patients. The presence of a zone 4 rearrangement was not associated with phase of disease, clinical evidence of acceleration, or pathologic evidence of acceleration. The difference between the actuarial duration of chronic phase for patients without (33 months) and with (22 months) a zone 4 rearrangement was not significant. A breakpoint in zone 4 of the bcr in patients with CML does not appear to be a determinant of prognosis.     

Exercise-induced hormone responses in girls at different stages of sexual maturation

The dependence of exercise-induced hormone responses on sexual maturation was tested in a 3-year longitudinal experiment on 34 girls (aged 11–12 years at the beginning). Sexual maturation was evaluated by Tanners five-stage scale. Children cycled for 20-min at 60% maximal oxygen uptake once a year. Cortisol, insulin, growth hormone, β-oestradiol, progesterone and testosterone concentrations in venous blood were determined by radioimmunoassay procedures. Basal concentrations of growth hormone increased and of cortisol decreased when breast stage III was reached. Reaching breast stage IV was associated with an increase in basal concentrations of β-oestradiol, progesterone and testosterone. The exercise induced significant increases in concentrations of cortisol, growth hormone and β-oestradiol and a decrease in insulin concentration. At breast stage III the increase in cortisol concentration was to a lower level [467 (SEM 42) vs 567 (SEM 46)nmol · l^?1] and growth hormone concentration to a higher level [29.4 (SEM 0.5) vs 12.8 (SEM 0.4)ng · ml^?1], while the fall in insulin concentration was less pronounced [postexercise level 10.6 (SEM 0.9) vs 7.8 (SEM 0.8)mU · l^?1] than in stage II. The magnitude of the cortisol response was reduced in the last stage of breast development (+42.1% vs +55.5% at stage II, +66.2% at stage III, and +50.0% at stage IV). The magnitude of β-oestradiol response was the lowest in breast stage IV (+15.8%) and the highest at stage V (+41.1%). The progesterone response became significant at stage IV and testosterone response at stage V. In conclusion, we found that reaching breast stage III was associated with altered responses of cortisol, insulin and growth hormone concentrations while the responses of the sex hormone concentrations became pronounced in the last stages of sexual maturation.     

Inflammatory myofibroblastic tumor of the pancreas with regional lymph node involvement

Inflammatory myofibroblastic tumors (IMT) can be found in virtually any location of the human body. Histologically a mesenchymal aspect predominates and makes these mostly benign tumors apt to be erroneously diagnosed as a soft tissue sarcoma. Cases showing infiltrative growth and local recurrence further complicate the assessment. Localization of an IMT in the pancreas is extremely rare. Clinical investigations regularly lead to the putative diagnosis of a malignant tumor and only subsequent histological examination can establish the correct tumor classification. We present the case of a 62-year-old woman with IMT of the pancreas. Evidence of lymph node involvement has not yet been reported in this setting.     

Expression of NK-associated antigens in extramedullary lymph nodal blast crisis of chronic myeloid leukemia on fine-needle cytology

A case is reported of a 62-yr-old male suffering from chronic myelogenous leukemia (CML) who developed an extramedullary, para-orthic lymph-nodal blast crisis without blood or bone marrow involvement and expression of CD56/NK associated marker. The diagnosis was performed on ultrasound-guided fine-needle cytology by an immunocytochemical and flow cytometric analysis. Conventional smears showed a monomorphous population of disperse, undifferentiated cells without cytoplasm. Cells showed fragile nuclei, vesicular chromatin, and evident nucleoli. Immunocytochemistry performed on cytospin slides were negative for cytokeratin, LCA, CD20, CD45Ro, and myeloperoxidase (MPO). Flow cytometry analysis proved the myeloid origin of the tumor by expression of CD13, CD34, and CD38 and showed aberrant expression of CD56. Cytological diagnosis was confirmed by histological examination. CD56 expression is generally an expression of NK lymphoid proliferation and may be observed in acute myelogenous leukemia but has rarely been reported in CML and its related blast crisis. This unusual expression, its possible explanation, the related technical problems, and clinicopathological aspects are discussed.     

不同成熟阶段树突状细胞的免疫耐受功能

大量研究证实具有抗原递呈作用的树突状细胞（DC）能够诱导免疫耐受，处于不同成熟阶段不同活化条件下的DC激活T细胞的能力不同。进一步研究发现DC产生的外染色体能够诱导T细胞免疫耐受，具有免疫原性。非成熟DC（iDC）产生的外染色体能够维持外周耐受，而成熟DC（mDC）产生的外染色体能够刺激效应性T细胞，因此外染色体在诱导免疫耐受中发挥着重要作用。     

New variant translocation (8;20)(q22;q13) in bone marrow cells of extramedullary blast crisis in chronic myelogenous leukemia

We used a novel RNase cleavage assay (NIRCA) to screen for neurofibromatosis 2 (NF2) mutations in NF2 schwannomas. Mutations were found in tumors in 16 of 20 patients. Eleven patients (55%) had loss of heterozygosity or loss of one allele, indicating that the mutation was a germ-line mutation. The phenotypes of these patients were consistent with previous NF2 genotype-phenotype correlation studies: patients with nonsense mutations had severe phenotypes, whereas those with splice-site or missense mutations had milder and variable phenotypes. These results confirm the utility of NIRCA as a rapid and convenient method for screening for germ-line NF2 mutations.