Anna Coci

Myelofibrosis in chronic granulocytic leukaemia: clinicopathologic correlations and prognostic significance

Summary. We performed 221 marrow trephine biopsies in 139 patients with Ph1‐positive (Ph1‐+) chronic granulocytic leukaemia (CGL) in order to assess the incidence, degree and prognostic significance of marrow fibrosis (MF) at various stages of the disease. We also attempted to elucidate the relationship between development of MF and the various clinical and haematological features of CGL. A significant correlation was found between the amount of fibrosis (graded from 0 to 3) and the stage of CGL, indicating that major fibrotic changes are associated with accelerated or blastic disease. Survival studies performed to assess the prognostic significance of the various degrees of MF, showed a progressively worse life‐expectancy from grade 0 to grade 3 fibrosis. Multivariate regression analysis indicated Hb level, age, number of marrow megakaryocytes (MKs), time from diagnosis as the features most significantly correlated with the degree of MF. This study demonstrates that the natural history of CGL involves a progressive increase in reticulin deposition towards severe MF, although the rate of this progression varies widely. Monitoring changes of fibrosis with sequential biopsies could give a measure of the rate of progression of the disease and help in prognostic assessment of CGL patients. Our findings also confirm that among marrow features the number of MKs is the cytological variable most significantly correlated with MF.

Risk assessment in myelodysplastic syndromes: value of clinical, hematologic and bone marrow histologic findings at presentation.

We analyzed the prognostic value of clinical, hematologic and bone marrow (BM) histologic findings at presentation in 94 patients with myelodysplastic syndromes (MDS) (28 RA; 2 RARS; 34 RAEB; 6 CMML; 24 RAEB‐t). With survival as the dependent variable, stepwise multivariate analysis indicated as the prognostically most important factors among the MDS taken as a whole: latency from the first symptoms to diagnosis, age, and percentage of BM blasts. In each main MDS group the most unfavorable initial characteristics were: l)low Hb, no macro‐megaloblastosis, male sex for RA/RARS; 2) low Hb and low platelet levels for RAEB/CMML; 3) granuloblastic hyperplasia and high BM blastosis for RAEB‐t. Of the BM histologic parameters, only the percentage of blasts had significant prognostic value. Histologic assessment of BM blastosis, however, did not differ statistically from that based on cytologic examination of BM smears, so that marrow histology seemed not essential for initial prognostic assessment in MDS patients. The finding of abnormal localization of immature precursors (ALIP) in BM biopsies was associated with a negative trend without reaching statistical significance. Using four objective parameters of proven significance (age, Hb, platelets, and BM blasts) we devised a staging system of immediate clinical utility for prognostic stratification and risk‐adapted therapeutic choices.

Bone marrow and blood involvement by non-Hodgkin's lymphoma: a study of clinicopathologic correlations and prognostic significance in relationship to the Working Formulation.

In a series of 172 patients with non‐Hodgkins lymphoma (NHL) classified according to the Working Formulation (WF) the overall incidence of bone marrow infiltration (BM +) at diagnosis was 39%: 59% for low‐grade (LGML), 30% for intermediate‐grade (IGML), and 25% for high‐grade malignant lymphomas (HGML). The features most significantly correlated with the presence of BM + were a low grade of histological malignancy, the degree of splenomegaly and high values of LDH, while those correlated with the extent of BM+ were a non‐focal pattern of BM disease, the presence of blood involvement at diagnosis, and the degree of BM fibrosis. Blood involvement was detected at diagnosis in 13% of patients, and a further 16% developed a leukemic phase during the course of the disease. Blood involvement correlated significantly with splenomegaly, bulky disease, advanced clinical stage, and extent of BM +. The presence of BM infiltration ‘per se’ at diagnosis did not significantly affect prognosis. However, the extent of BM disease was correlated with a poorer outcome in IGML and HGML patients. Regarding peripheral blood involvement, in LGML patients only late leukemic conversions were significantly associated with a worse prognosis. In patients with IGML and HGML, either initial or subsequent blood involvement was correlated with significantly poorer outcome.

Inhibitory effect of salmon calcitonin on bone resorption: Morphological study of the tibial growth plate in rats

SummarySalmon calcitonin (sCT) at doses of 100 and 50 UI given subcutaneously to growing rats produced in vivo evidence of osteoclastic activity inhibition. Histological assessment was carried out by measuring the perichondrial ring of Lacroix height, and a dose-correlated effect was found. These aspects were coupled with an increase in the osteoclast number and suggested that in studies with bone resorption inhibitors, morphological evaluation based on osteoclasts count is not reliable. The changes of the metaphysis suggested also that sCT affects the activity of hypertrophic chondrocytes of the growth plate. Plasma calcium levels did not differ significantly between treated rats and controls; an increased phosphatemia was observed in sCT-treated animals.

Bone changes of mucolipidosis II at different ages : postmortem study of three cases

Bone changes are a constant feature of mucolipidosis II, with striking differences between newborns and older children. Intracellular, membrane-bound vacuoles were found in the chondrocytes, osteoblasts, osteocytes, and stromal fibroblasts of three affected children. Osteoclasts and marrow cells were unaffected. Ricketslike lesions were present at birth in the two younger cases, whereas signs of high bone turnover and defective calcification were no longer present in the older child. Severe abnormalities of the metaphyseal plate with the loss of normal cartilage architecture and the absence of endochondral ossification were the major changes in this age group.

Bone marrow and blood involvement by non-Hodgkin’s lymphoma: Clinicopathologic features and prognostic significance in relationship to the Working Formulation

Bone marrow (BM) biopsy is a valuable staging procedure for patients with malignant non-Hodgkin’s lymphomas (NHL). In cases presenting with nodal involvement, a positive bone marrow finding would allow diagnosis of Stage IV disease without the need for more aggressive procedures. Previous studies have evaluated the frequency and pattern of bone marrow involvement in the various pathologic subtypes of the NHL classified according to the Rappaport [1–5], Lukes and Collins [6, 7], and Kiel [8–11] schemes. Furthermore, the results of Bartl’s study [10] applying the Kiel classification to BM biopsies, indicated the prognostic significance of BM disease in NHL. As concerns peripheral blood involvement by malignant lymphomas, the leukemic phase has been the subject of several studies, all based on the Rappaport [12–14], Lukes and Collins [7], WHO [15, 16] and Kiel systems [17].

Malignant small cell tumor of the thoracopulmonary region in childhood: a case report

A case of malignant small cell tumor of the thoracopulmonary region in a 3-year-old boy is presented. The rarity of this tumor in children justifies the presentation of a new case. Differential diagnosis with other childhood neoplasms is discussed.