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M Zandecki

Publications and source records attributed to M Zandecki.

At least 73 records · Page 4Linked to original sources
PubMed1989

Cytological and ultrastructural assessment of free crystals or precipitates associated with pseudoleukocytosis and pseudothrombocytosis in cryoglobulinemia.

In 3 patients with cryoglobulinemia (case 1: type I: cases 2 and 3: type II) spurious leukocyte and platelet counts were seen using automatic particle counters, with up to 2.5X and 3X counts for leukocytes and platelets respectively, with peculiar volume histograms. All these anomalies were absent with manual counting and were still evident in warmed blood samples from 1 of the 3 patients. The corresponding blood smears showed numerous deposits, rectangular in the type I cryoglobulinemia and flake-like in the other two (type II) patients, responsible for the erroneous automatic counting. Ultrastructural study showed in cases 2 and 3 they consisted of dense amorphous protein clusters; and in case 1, a periodic disposition of hollow rods (each with 21-24 nm external diameter). Comparison with ultrastructure descriptions of isolated cryoglobulins from the literature showed that crystals made of hollow rods might be preferentially associated with monoclonal GK immunoglobulin. Close scrutiny of histograms from automatic cell counters and stained blood films is necessary to detect falsely elevated counts in patients with cryoglobulinemia, and in the management of suppressive therapy.

Adult↗
PubMed1989

Cytogenetic studies in 30 patients with Burkitt's lymphoma or L3 acute lymphoblastic leukemia with special reference to additional chromosome abnormalities.

We performed cytogenetic analysis in 23 consecutive patients with Burkitt's ALL and 7 patients with Burkitt's lymphoma. Only one patient had a normal karyotype. Twenty-seven patients had a (8;14) translocation and 2 a (2;8) translocation. No (8;22) translocation was seen. In 12 patients (41%), the t(8;14) was the only chromosome rearrangement whereas in the 18 remaining cases (59%), the t(8;14) or t(2;8) was associated with other numerical or structural abnormalities. Chromosomes 1, 7 and 6 were rearranged in 10, 8, and 5 patients, respectively, usually in translocations, duplications, deletions (chromosome 6), or isochromosome of the long arm (chromosomes 1 or 7). The incidence of these additional rearrangements is discussed with regard to previously published reports and the chromosome localization of oncogenes.

Burkitt Lymphoma↗
PubMed1988

Acute monoblastic leukemia with osteosclerosis and extensive myelofibrosis.

A 4-month-old infant was admitted with a monoblastic infiltration of the skin associated with osteosclerosis. Both lesions spontaneously disappeared within a few months, but 2 years later, a monoblastic leukemia occurred that was associated with marked skin erythema and myelofibrosis. Skin and bone marrow specimens showed a monoblastic infiltration with numerous intermingled mast cells of normal appearance. Whether myelofibrosis was a feature of a systemic mastocytosis or of the leukemic process is discussed in this case.

Humans↗
PubMed1988

Refractory anaemia according to the FAB classification: a report on 69 cases.

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.

Adult↗
PubMed1988

Congenital acute monoblastic leukemia with double translocation (8;16) (p11;p13) and (16;20) (q13;p13).

A new case of congenital acute monoblastic leukemia (AML5) with a rare (8;16) translocation is described: this translocation (or a variant) was previously reported only in 4 infants and 10 older children or adults, practically always in association with AML5 or acute myelomonocytic leukemia. The authors discuss the possible causative implication of band 8p11 with some cytological particularities, i.e. erythrophagocytosis by some blasts and strong peroxidase and esterase activities, observable here and in most of the cases with the t(8;16). The t(8;16) may be added to the other chromosomal abnormalities of congenital AML5.

Chromosome Banding↗
PubMed1987

Three cases of translocation (8;16)(p11;p13) observed in acute myelomonocytic leukemia: a new specific subgroup?

In three cases of acute nonlymphocytic leukemia we observed a translocation (8;16)(p11;p13); in one case it was the sole karyotypic change and in the other two cases it was associated with other structural anomalies. All three cases were nonhyperleukocytic myelomonocytic leukemias with erythrophagocytosis by some blast cells and cytochemistry results consistent with leukemic proliferation of a common monocytic-granulocytic precursor. The importance of this translocation is discussed, and the implication of band 16p13 in myelomonocytic leukemia is stressed.

Adolescent↗
PubMed1987

Chronic and subacute myelomonocytic leukaemia in the adult: a report of 60 cases with special reference to prognostic factors.

We report 60 cases of chronic and subacute myelomonocytic leukaemias (CMML and SMML) in the adult, using the FAB group criteria. The M/F sex ratio was 3.3 and the mean age 67.5 years. Splenomegaly was found in 32% of cases, hyperleucocytosis in 52% of cases and mean blood monocytosis was 4.3 X 10(9)/l. Marrow smears showed an excess of blasts in 57% of patients, a moderate increase in monocytes in most cases and frequent myelodysplastic features. An increase in serum lysozyme and polyclonal hypergammaglobulinaemia were usual and clonal cytogenetic anomalies found in about half of the patients tested. Treatment was usually palliative and the median survival was 28 months, a blastic transformation being responsible for a third of the deaths. Prognostic factors at diagnosis were analysed retrospectively in the 46 patients who had sufficient follow up. Percentage of marrow blasts haemoglobin level and blood monocytosis at diagnosis, were subject to multivariate analysis, resulting in a discriminant 'score'. This allowed assignment of each patient into one of two prognostic subgroups (10.9% probability of error): a poor prognosis one, with a life expectancy of less than 1 year and a high risk of acute transformation (subgroup termed SMML) and a better prognosis subgroup (termed CMML), with some CMML patients surviving over 5 years.

Adult↗
PubMed1987

Total peroxidase deficiency in eosinophils: a report on twin sisters, one with a refractory anaemia.

A total lack of EPO was fortuitously discovered in a 63-yr-old woman from the north of France who had primary refractory anaemia, but without dysgranulopoiesis; MPO activity was normal. Her twin sister, whose blood count was normal, also had EPO deficiency. This familial disorder was first described in the Israeli Jewish population and is very rare in Caucasians; it seems to have no pathological consequences. Microscopic studies showed no anomaly except a negativity of Sudan Black B staining which we consider to be a sign of peroxidase deficiency. Ultrastructural studies of the granules revealed normal cristalloid, but the matrix, which contains EPO in normal eosinophils, was very thin; the nature of the relation between functional and morphological anomalies has still to be elucidated.

Anemia↗
PubMed1987

[Cytological diagnosis of myelodysplastic syndromes. Apropos of 200 cases].

The authors present a cytological study of 200 cases of myelodysplastic syndromes (MDS) [corrected], classified according to the FAB cooperative group. This analysis involves the conventional parameters of the peripheral blood and bone marrow differential counts, the systematic recording of signs of dysmyelopoiesis on the erythroblastic [corrected], granulocytic and megacaryocytic lineages and the assessment of blood granulocytes myeloperoxidase (MPO). On the outside of the typical acquired idiopathic sideroblastic anaemia (severe, isolated anaemia associated with intense dyserythropoiesis), the diagnosis of MDS requires very astute cytological interpretation: indeed, when the dysgranulopoiesis is pronounced, determination of peripheral blood and bone marrow differential count is especially difficult. Conversely when the dysmyelopoiesis is slight, a systematic search of morphology signs [corrected] of its presence must be done and associated with functional studies such as MPO activity technically easy to realize.

Adult↗
PubMed1987

[Acute lymphoblastic leukemia, Burkitt-cell type, after Hodgkin's disease. Study of 2 cases].

Two cases of Burkitt cell leukaemia (ALL3) occurring 60 and 51 months respectively after treatment of Hodgkin's disease (HD) are described. Clinical, cytological, immunological and cytogenetic features in these patients were comparable with those of the 6 previously published cases and with de novo ALL3. The prognosis of these secondary ALL3 is uniformly poor: our 2 patients died 2 and 14 days after the start of combination chemotherapy, and all the other cases survived less than 60 days after diagnosis. The significance of ALL3 occurring after HD is discussed: the association is probably not fortuitous, and its pathogenic mechanisms probably differ from those intervening in secondary myeloid leukaemias.

Adult↗
PubMed1987

[Ineffective erythropoiesis of immunologic origin with free bilirubin jaundice in an elderly subject].

We report on a 74-year-old male patient who presented with jaundice, highly increased unconjugated bilirubin and slight nonregenerative anemia. Ferrokinetic studies demonstrated ineffective erythropoiesis and red cell survival, studied by 51 Cr, showed moderately increased hemolysis. Other findings showed no evidence of an underlying myelodysplastic syndrome but strongly favored the autoimmune origin of the disorder, with immunologic destruction of erythroblasts (and to a far lesser extent erythrocytes) by autoantibodies.

Aged↗
PubMed1987

[(9;11) (p21;q23) translocation: apropos of 2 new cases seen in acute undifferentiated monoblastic AML5a leukemia].

In a cytogenetic study of 54 patients with acute monoblastic leukaemias (AML5) two had a (9;11) (p21;q23) translocation. In addition to the clone, with t(9;11), both patients had a second clone with t(9;11) and trisomy 8. These two patients were aged 23 and 35 years at diagnosis and were classified as M5a in the FAB formulation. A complete remission was achieved in each case, persisting after 19 and 18 months respectively (autograft for one patient). Whereas chromosome 11 anomalies are involved in 13% of all AML5 cases, t(9;11) is less frequently encountered (3.7% in our experience).

Adult↗
PubMed1986

[Hemophagocytic syndrome associated with herpes simplex virus. Apropos of a case with a fatal outcome].

A male patient, aged 23 years, with no notable previous history, was admitted to hospital because of fever, splenomegaly, and pancytopenia. The bone marrow was infiltrated by mature looking histiocytes displaying erythrophagocytosis. Other findings included polyclonal hypergammaglobulinemia, elevated transaminases, hypofibrinogenemia, and hypertriglyceridemia. A diagnosis of virus-associated hemophagocytic syndrome was made and virology studies attributed it to herpes simplex virus type 1. Therapy with acyclovir, high dose i.v. immunoglobulins, and vinblastine was unsuccessful. With the addition of prednisone, a remission, with disappearance of histiocytic marrow involvement, was obtained but was of short duration, and the outcome was fatal.

Adult↗
PubMed1983

[Transient leukoblastosis and dysmegakariocytopoiesis with clone 46, XX+21, t(5;7), in a newborn infant with trisomy 21].

A case of transient leukemoid reaction in a child with Down's syndrome with the presence of leukoblasts in the blood at birth is reported. The karyotype established on culture of lymphocytes and fibroblasts was characterized by a chromosomic formula 47, XX,21+. The karyotype established on day 13 of life on cultured bone marrow showed a trisomic 21 abnormal clone with 46 chromosomes resulting from a translocation of the long arms of chromosomes 5 and 7, which gave the chromosomic formula: 46, XX, -5, -7,t (5 qter leads to cen leads to 7 qter), +21. This clone was present in spontaneous blood mitoses. It disappeared on day 75 of life as well as the abnormal leukoblasts. The peculiarity of this case in due to the presence of an aneuploid clone and the association of a leukemoid reaction and dysmegakaryocytopoiesis both of which were transient.

Chromosome Aberrations↗
PubMed1983

[Transitory leukemoid reaction with regressive clonal course in a mongoloid newborn infant].

A neonate with Down's syndrome presented with partial blast infiltration that spontaneously regressed without relapse at age 6 months. Sequential cytogenetic studies of cells in blood and bone marrow initially showed presence of a clone with 46 chromosomes and reciprocal translocation of chromosomes 5 and 7. This clone and the hematologic abnormality later disappeared simultaneously. Two explanations may be suggested: a defect in the maturation of normal blasts due to abnormal environment in the bone marrow, or an intrinsic abnormality of hematopoietic cells. The case reported would favor the second hypothesis.

Chromosomes, Human, 4-5↗
PubMed1983

[Burkitt's acute lymphoblastic leukemia. Incidence and cytogenetic aspects].

We report 4 cases of patients, 5, 16, 31 and 55 years old respectively, with Burkitt type ALL. Three of them presented with a hypoaesthesia of the chin tip. The cytological features of abnormal cells were those of L3 subgroup in the FAB classification; they expressed surface immunoglobulin markers. EBV serology was positive in two cases but the titres did not indicate a recent infection. The translocation t(8;14) (q24;q32) was found in all 4 patients. In one case, it was possible to characterize a clone with a 7 neochromosome (7p+) and a clone with a supernumerary 7 isochromosome i(7q)+; in other respects these two clones showed the translocation t(8;14) and only the latter remained in the terminal phase. In these 4 patients the disease progressed dramatically even when haematological remission was obtained (two cases), predominantly due to early neuro-meningeal involvement.

Adolescent↗