Inter-observer variance with the diagnosis of myelodysplastic syndromes (MDS) following the 2008 WHO classification.

Morphology is the basis of the diagnosis of myelodysplastic syndromes (MDS). The WHO classification offers prognostic information and helps with the treatment decisions. However, morphological changes are subject to potential inter-observer variance. The aim of our study was to explore the reliability of the 2008 WHO classification of MDS, reviewing 100 samples previously diagnosed with MDS using the 2001 WHO criteria. Specimens were collected from 10 hospitals and were evaluated by 10 morphologists, working in five pairs. Each observer evaluated 20 samples, and each sample was analyzed independently by two morphologists. The second observer was blinded to the clinical and laboratory data, except for the peripheral blood (PB) counts. Nineteen cases were considered as unclassified MDS (MDS-U) by the 2001 WHO classification, but only three remained as MDS-U by the 2008 WHO proposal. Discordance was observed in 26 of the 95 samples considered suitable (27 %). Although there were a high number of observers taking part, the rate of discordance was quite similar among the five pairs. The inter-observer concordance was very good regarding refractory anemia with excess blasts type 1 (RAEB-1) (10 of 12 cases, 84 %), RAEB-2 (nine of 10 cases, 90 %), and also good regarding refractory cytopenia with multilineage dysplasia (37 of 50 cases, 74 %). However, the categories with unilineage dysplasia were not reproducible in most of the cases. The rate of concordance with refractory cytopenia with unilineage dysplasia was 40 % (two of five cases) and 25 % with RA with ring sideroblasts (two of eight). Our results show that the 2008 WHO classification gives a more accurate stratification of MDS but also illustrates the difficulty in diagnosing MDS with unilineage dysplasia.

[Basophilic blast crisis in chronic myeloid leukemia]. / Crisis blástica basófila de leucemia mieloide crónica.

Acute basophilic leukaemia is usually characterized by a very rapid clinical course, hyperhistaminemia, resistance to antineoplastic therapy and early death due to complications related to disease. This entity is a rare condition, accounting for less than two percent of all haematopoietic malignancies. Most of the case reports are basophilic blast crisis in patients with a previous lympho or myeloproliferative disorder. A 62-year-old woman who was diagnosed as Philadelphia positive chronic myelogenous leukaemia after four years of evolution developed a basophilic blast crisis, whose characteristics are reported. Accompanying this transformation there was also a cytogenetic change. Despite chemotherapy the patient died of disease progression.

[Vasculitis associated with a myelodysplastic syndrome: a report of 5 cases]. / Vasculitis asociada a un síndrome mielodisplásico: aportación de 5 casos.

In a study of 94 patients with myelodysplastic syndrome (MDS) associated vasculitis was observed in 5, which preceded hematologic diagnosis in 4. Leukocytoclastic vasculitis was observed in 5 cases being associated to lobular panniculitis in one. Three patients had refractory anemia, one sideroblastic anemia (SA) and another refractory anemia with excess blasts (RAEB). In the latter case lymphomatoid papulosis was also observed which, to date, has not been previously described in association with MDS. Another case presented seronegative polyarthritis and renal disease coinciding with vasculitis. Polyarteritis nodosa was diagnosed in a third patient in agreement with the criteria of the American College of Rheumatology, the association of which with MDS is exceptional. In the same case medullary cytogenetic study showed 46, XY,t (12;20), an abnormally which has not been described to date in cases of MDS. All the cases were treated with glucocorticoids in addition to cyclophosphamide in the patient with polyarteritis nodosa, with an improvement in the vasculitis being observed in all the patients. Two patients died, one (SA) due to pneumonia and the other (RAEB) due to subdural hematoma following transformation to acute myeloblastic leukemia. With 5% of the MDS studied presenting vasculitis, this syndrome should be included in the differential diagnosis of vasculitis observed in patients with cytopenia.

Cytohematologic and cytogenetic prognostic factors at diagnosis and in the evolution in 46 primary myelodysplastic syndromes.

The myelodysplastic syndromes (MDS) are a heterogeneous group of diseases with different prognosis and evolution. Most of the studies on prognostic factors performed previously have independently evaluated the clinico-hematologic or cytogenetic data at diagnosis. In the present paper, 46 primary MDS were clinically, hematologically, and cytogenetically investigated at diagnosis, in order to determine the principal factors affecting the survival probability between a great number of characteristics. A univariate regression analysis of all the data allows one to recognize that the main factors are: the complexity of karyotype (p = 0.00001), the percentage of type I and total marrow blast cells (p = 0.001), and the abnormal localized immature myeloid precursors' (ALIP) presence (p = 0.001). Twenty-five patients underwent consecutive studies during their evolution. The karyotype instability gives information both on the likely evolution to acute leukemia and on poor survival.

[Usefulness of cytometry based on the diffraction of a laser beam in evaluating spherocytosis]. / Utilidad de la citometría basada en la difracción de un haz de luz láser en la valoración de la esferocitosis.

Thirty four cases of hereditary spherocytosis were studied by means of laser diffraction cytometry. The cases were grouped for study in accordance to previous splenectomy or not, familial involvement or not, and, in patients not subjected to splenectomy, severity of the clinical course. The values used to assess the presence of spherocytosis were those measuring the haemoglobin concentration within red cells, such as CH (directly estimated mean corpuscular haemoglobin), HDW (standard deviation of the distribution according to haemoglobin concentration) and % hyper (percentage of cells with haemoglobin concentration higher than 41 g/dL). The variables attained were statistically analysed by means of non-parametric tests. In patients with spherocytosis, MCV, MCHC, RDW, HDW, % hyper, and CH were significantly different from the normal group. This method points to the presence of spherocytosis by means of CH, HDW and % hyper. A reduction of the limit of haemoglobin concentration used to define % hyper (41 g/dL) could improve the sensitivity of the instrument for the diagnosis of the mild forms, which is often more difficult.

Hemophagocytic syndrome causing complete bone marrow failure. Report of an extreme case of a reactive histiocytic disorder.

The hemophagocytic syndrome is a reactive disorder of the mononuclear phagocytic system. Most of the cases are rare complications of common infectious and neoplastic diseases, although there may be an underlying immune disorder predisposing to this syndrome. We report a case in association with immune thrombocytopenia and hemolytic anemia (Evans' syndrome). The hemophagocytic reaction appeared after a bacterial infection of the urinary tract and presented with abrupt pancytopenia and complete hemopoietic failure. We discuss the possible mechanisms of bone marrow failure related with the hemophagocytic syndrome.

Lymphoblastic lymphoma/leukemia of T-cell origin: ultrastructural, cytochemical, and immunologic features of ten cases.

Ten cases of T-lymphoblastic lymphoma/leukemia were studied with light and electron microscopy. Cytochemical strains were performed on touch preparations, and mononuclear cell suspensions were tested for spontaneous rosette formation with sheep erythrocytes, C3 receptors, and surface immunoglobulins. The present investigation was performed to evaluate several ultrastructural parameters, mainly the nuclear shape, as diagnostic clues for this group of lymphomas. Characteristic convoluted nuclei were present in 7 to 47% of the lymphoblasts. This percentage correlated with the focal acid phosphatase reaction and E-rosette formation. Acid phosphatase was the best cytochemical marker (70-100% of the lymphoblasts showed focal reaction product). By ultrastructural cytochemistry, the reaction product was demonstrated in the Golgi cisternae and primary lysosomes. The cell suspensions obtained from different sources contained 14 to 95% E-rosette-forming cells. No specific morphologic, cytochemical, or immunologic differences were found between patients with or without mediastinal involvement.