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Biomedical subjects

C Rozman

Publications and source records attributed to C Rozman.

At least 163 records · Page 9Linked to original sources

[Chronic myeloid leukemia after chemotherapy treatment for non-Hodgkin's lymphoma].

A 52 year old male presenting chronic myeloid leukemia (CML) Philadelphia chromosome positive (Ph) four years after the diagnosis of a non Hodgkin's lymphoma is described. The patient had received high total doses of alkylating drugs (cyclophosphamide and chlorambucil) as part of chemotherapy treatment for a diffuse mixed lymphoma. At four years of diagnosis of the lymphoma the appearance of hepatosplenomegaly, leukocytosis with myeloma and basophilia and thrombocytosis were observed. These alterations augmented progressively until a cytogenetic study of the bone marrow two years late established the diagnosis of CML upon demonstrating the presence of the Ph chromosome with no other karyotypic anomalies being observed. The explorations carried out at that time confirmed that the lymphoma continued to be in remission. The CML initially responded to treatment with busulphan. However, following a year and a half the disease evolved to a phase of acceleration and the patient died a few weeks later due to pneumonia with no signs indicative of lymphoma activity having been detected since the diagnosis of the CML.

Alkylating Agents↗

[Behçet's disease with an onset prior to the appearance of chronic myeloid leukemia].

The case of a Ph-positive female patient with chronic myeloid leukemia (CML) is reported. The patient presented a cutaneous-mucous picture prior to the appearance of the hemopathy consisting of genitals ulcers, buccal aphthae and nodular cutaneous lesions the study of which demonstrated panniculitis. The lesions improved with the administration of low doses of prednisone and colchicine. The CML evolved to a blastic crisis of a monocytic phenotype at 14 months of diagnosis leading to death of the patient. The cutaneous-mucous picture was catalogued as Becçet disease (BD) according to the criteria of the International Study Group for Behçet Disease. Given the lack of serologic tests or pathognomonic histologic lesions the difficulty in the diagnosis of BD is commented upon and the differential diagnosis of this disease, particularly with respect to the Sweet syndrome, is discussed.

Behcet Syndrome↗

Dyserythropoiesis in iron-deficiency anemia: ultrastructural reassessment.

Iron deficiency is usually included among the causes of acquired dyserythropoiesis. This concept was derived mainly from light microscopic studies. To reassess such a notion at ultrastructural level, a transmission electron microscopic evaluation of bone marrow was performed in seven patients with iron-deficiency anemia. In contrast to the widely accepted concept, derived from light microscopic studies, only a small proportion (2-4%, not different from controls) of erythroblasts displayed some of the features of nuclear dyserythropoiesis. On the contrary, when examining the cytoplasm, we found a significantly increased number of void ropheocytotic vesicles in the majority of late erythroblasts as compared to controls (P less than 0.001). This feature may be considered as an ultrastructural marker of iron deficiency and is consistent with the present knowledge on transferrin-mediated delivery of iron to the cell.

Adolescent↗

Chronic systemic candidiasis in acute leukemia.

In the past few years a new syndrome of invasive Candida infection, the so-called hepatosplenic or chronic systemic candidiasis (CSC), has been recognized with increasing frequency in neutropenic patients. From January 1985 to December 1990, ten of 305 acute leukemia (AL) patients treated at our institution were diagnosed as having CSC. In contrast, during the same period this type of Candida infection was not observed in any patient with hematological diseases other than AL treated in our center, including 277 patients who underwent bone marrow transplantation. All patients with CSC had fever and hepatomegaly, and five complained of abdominal pain. Seven patients had neutrophilic leukocytosis and six an increased serum alkaline phosphatase activity. Abdominal computed tomography and ultrasound study showed typical lesions in eight and seven patients, respectively. In four patients a laparoscopy-guided needle liver biopsy displayed yellowish nodules on the liver surface, and the histologic study revealed large granulomas with yeasts and pseudohyphae. All patients were given amphotericin B (mean: 4.6 g, range: 1-12.5 g) and 5-fluorocytosine, and five received fluconazole. No patient died as a direct consequence of CSC and in six the infection resolved. Finally, once controlled, the infectious complication did not preclude subsequent intensive antileukemic therapy, including bone marrow transplantation.

Adolescent↗

Malignant transformation and life expectancy in monoclonal gammopathy of undetermined significance.

The actuarial probability of malignant transformation and the impact on expected survival were analysed in a series of 128 persons diagnosed with monoclonal gammopathy of undetermined significance (MGUS) over a 20-year period. At a median follow-up of 56 months the M-component remains stable in 101 patients (78.9%), 14 patients (10.9%) have died from non-related disorders and 13 (10.2%) have developed malignant transformation of MGUS (multiple myeloma, 10; primary amyloidosis, two; Waldenström's macroglobulinaemia, one). The actuarial probability of malignant transformation at 5 and 10 years was 8.5% and 19.2%, respectively. When different presenting features were analysed for predictive value of the malignant transformation, the IgA type of MGUS was the only variable associated with a higher probability of such an event (P less than 0.025). Although no significant difference was observed between the survival probability of persons with MGUS and that of the control population, the development of malignant transformation was associated with a shorter survival (P less than 0.001).

Adult↗

Neutrophilic pustulosis associated with chronic myeloid leukemia: a special form of Sweet's syndrome. Report of two cases.

Two subjects with Ph-positive chronic myeloid leukemia (CML) in whom pustular Sweet's syndrome was diagnosed are reported. The first patient was a 47-year-old woman who developed fever, painful ulcers of the oral mucosa and vagina and generalized pustulous skin lesions 2 years after the diagnosis of CML. Histologically, the skin lesions consisted of dense neutrophilic infiltrates with perifollicular disposition. The microbiologic studies were negative. The lesions showed a favorable response to corticosteroids, but fever recurred with every attempt of tapering prednisone; it finally disappeared with the addition of oral cyclophosphamide. The second patient was a 45-year-old man who developed fever and disseminated pustules with histologic features consistent with Sweet's syndrome and negative microbiologic studies at 2.5 years after diagnosis of CML. The picture showed a dramatic response to prednisone and did not recur after the drug was discontinued. In both patients, CML remained stable after resolution of Sweet's syndrome.

Cyclophosphamide↗

An unusual case of Castleman's disease restricted to the neck.

An unusual case of Castleman's disease (CD) restricted to the left cervical area is reported. CD is rarely confined to the cervical area; when it occurs, it is usually of the hyaline-vascular subtype and small in size. This case was atypical due to its histology (plasma-cell variant), big size (15 x 11 x 6 cm) and the absence of general symptoms. In spite of surgical excision, the disease recurred 11 months later. However, subsequent surgery followed by chemotherapy resulted in a long-lasting control of the disease.

Adult↗

Polycythaemia vera following non-Hodgkin's lymphoma.

A patient with non-Hodgkin's lymphoma (NHL) who developed polycythaemia vera (PV) is reported. Diffuse large-cell NHL was diagnosed and he was subsequently treated with combination chemotherapy including high dose cyclophosphamide and procarbazine. Four and a half years after chemotherapy splenomegaly developed, coincidently with the appearance of high Hb values, RBC and platelet counts. The screening tests for PV were consistent with this diagnosis, while the search for lymphoma activity was negative. To the best of our knowledge, the present case represents the first well-documented instance of PV following NHL.

Humans↗

[Stereological study of the fat cells in bone marrows with a heterogeneous distribution of adipose tissue].

PURPOSE: 1) To analyze to what extent the fat tissue fraction of the human bone marrow with heterogeneous distribution depends on size and number of adipocytes. 2) To infer the influence of local factors on the two aforementioned parameters. MATERIAL AND METHODS: The material was made up of 15 specimens of bone marrow biopsy with markedly heterogeneous distribution of fat tissue, alternating normal or hyperplastic zones (area I) with aplastic ones (area II). The method of study was the stereological technique on plastic-embedded specimen sections. RESULTS: In the area II, with a fat tissue fraction markedly higher than in area I, both adipocyte number and size were significatively increased. The fat tissue fraction difference (Dif FRGR) between both areas was significatively correlated with the difference of the adipocytes number (Dif Nv) but not of the size (Dif D). However, in multiple regression both Dif Nv and Dif D contributed significatively to Dif FRGR. CONCLUSIONS: Since the histopathological pattern investigated in this work represents a model caused by intervention of local factors, it can be concluded that these can modify both the size and number of adipocytes.

Adipose Tissue↗

Recombinant alpha-2b-interferon may restore natural-killer activity in patients with B-chronic lymphocytic leukemia.

In nine patients with CLL treated with chlorambucil followed by alpha-2b-interferon (alpha 2b-IFN), T, B and natural killer (NK) cells and NK activity were studied before entering the study, after chlorambucil treatment, and after administration of alpha 2b-IFN. When considered as a whole, basal NK activity was lower in CLL patients than in controls (21.0% +/- 10.9 versus 40.2% +/- 17.4, p less than 0.001); however, when considered individually, four out of nine patients had normal NK activity at diagnosis. Chlorambucil did not increase global NK activity (21.7% +/- 7.1), whereas alpha 2b-IFN did so (44.3% +/- 19.1). After alpha 2b-IFN only one of seven patients studied had low NK activity. Previously increased absolute counts of CD2+, CD4+, CD8+, CD16+, CD57+ lymphocytes were reversed with chlorambucil treatment to normal levels, while after this therapy CD11b+ and CD19+ cells decreased without reaching normal values. During alpha 2b-IFN therapy, an increase up to normal levels in the percentage of CD16+ (2.7% +/- 3.4 versus 7.7% +/- 6.5, p = 0.04) and CD57+ (3.0% +/- 3.0 versus 8.1% +/- 6.2, p = 0.020) lymphocytes was observed whereas the absolute number of CD19+ B-cells further decreased (5.2 x 10(9)/l +/- 2.5 vs 3.8 x 10(9)/l +/- 2.3), albeit not significantly.

Adult↗

[Meningeal infiltration in multiple myeloma. Study of a new case and literature review].

A patient with lambda light-chains Bence-Jones multiple myeloma (MM) showed a meningeal myelomatosis during a relapse of his illness. Meningeal infiltration was showed through the detection of plasmatic cells in cerebro spinal fluid, identified morphologic and immunophenotypically, together with hyperproteinemia constituted exclusively by lambda light-chains. Treatment was given, intrathecal (methotrexate and cytosine arabinoside) and systemic (vincristine, adriamycin and dexamethasone) chemotherapy, with disappearance of meningeal infiltration. However the patient died, after three months evolution of MM, tough. Literature on this topic is reviewed.

Adult↗

[Granulocyte alkaline phosphatase activity in the chronic phase and blastic crisis of chronic myeloid leukemia. Sequential study of 43 patients].

PURPOSE: To evaluate in a sequential fashion the activity of the leukocyte alkaline phosphatase (LAP) in the chronic phase and the blastic crisis of chronic myelogenous leukaemia (CML). MATERIAL AND METHODS: This study is comprised of 43 patients diagnosed of CML according to standard criteria. The initial LAP scores were compared with those recorded in the blastic crisis, using cytochemical methods. The statistical analysis was performed with Student's test and chi-square. RESULTS: The LAP activity at diagnosis was low in 40 of the 43 cases (93%), the score being 0 in 20 instances. In the blastic crisis low scores were found in 27 patients (63%), while LAP activity appeared normal or increased in 16 others (37%). When the LAP scores of the two phases of the disease were compared the differences were found statistically significant (p less than 0.0001 and p less than 0.0007, respectively). CONCLUSIONS: These results confirm that the onset of the blastic crisis of CML is often accompanied by an increase of the LAP activity, although this last persists low in the majority of the patients.

Adolescent↗