Subject(s)
Erythrocytes/chemistry , Membrane Proteins/deficiency , Spherocytosis, Hereditary/etiology , Anion Exchange Protein 1, Erythrocyte/deficiency , Ankyrins/deficiency , Erythrocytes/metabolism , Family Health , Humans , Reticulocytes/chemistry , Reticulocytes/metabolism , Spain/epidemiology , Spectrin/deficiency , Spherocytosis, Hereditary/epidemiology , Spherocytosis, Hereditary/metabolismABSTRACT
We have applied dual angle laser scattering cytometry (DALC), which provides objective assessment of spherocytosis, to study the changes of hereditary spherocytosis (HS) red cell populations after splenectomy. Eighty unsplenectomized HS patients (32 mild, 37 moderate and 11 severe cases), 26 splenectomized HS patients (SHS, formerly 21 moderate and five severe cases) and 140 controls were studied. SHS were similar to unsplenectomized mild HS cases, with homogeneous red cell volume distribution and spherocytosis. Spherocytosis in SHS was significantly higher when compared with controls (P < 0.001) but less when compared with unsplenectomized HS patients (P < 0.01).
Subject(s)
Flow Cytometry/methods , Hemoglobinometry/methods , Spherocytosis, Hereditary/surgery , Splenectomy , Adolescent , Child , Erythrocyte Indices , Hemoglobins/analysis , Humans , Lasers , Spherocytosis, Hereditary/physiopathologyABSTRACT
Dual angle laser scattering cytometry (DALC) measures erythrocyte volume and haemoglobin concentration directly and provides spherocyte quantification. We have studied DALC red cell features in hereditary spherocytosis (HS) relating them to the clinical severity of the illness. A total of 140 controls and 80 non-splenectomized HS cases were studied, with mild (n = 32), moderate (n = 37) and severe (n = 11) disease. DALC measured mean corpuscular haemoglobin concentration, percentage of erythrocytes with haemoglobin concentration over 410 g/l (%Hyper), red cell volume and haemoglobin concentration distribution widths which were significantly different in HS cases when compared with controls (P < 0.001). Severe HS cases had more heterogeneous erythrocyte populations, both in volume and haemoglobin concentration (P < 0.001). Clinical severity of HS was best reflected by erythrocyte volume distribution width followed by %Hyper. DALC red cell features are both a diagnostic tool and an index to the severity of HS.
Subject(s)
Flow Cytometry/methods , Hemoglobinometry/methods , Spherocytosis, Hereditary/blood , Adolescent , Adult , Aged , Child , Child, Preschool , Discriminant Analysis , Erythrocyte Volume , Female , Humans , Infant , Lasers , Male , Middle Aged , Severity of Illness IndexSubject(s)
Aspartic Acid/chemistry , Glycine/chemistry , Hemoglobin J/genetics , Heterozygote , beta-Thalassemia/genetics , Adolescent , Base Sequence , Child , Humans , Male , Molecular Sequence Data , SpainABSTRACT
A 32-year-old male developed severe autoimmune thrombocytopenia refractory to conventional immunosuppression. He had been treated with radiotherapy for stage I-A Hodgkin's disease (HD) 2 years earlier after a staging laparotomy and splenectomy. A 3-cm accessory spleen was detected using computed tomography scan and 99mTc scintigraphy. Resection resulted in normalization of the platelet counts. Two years later the patient remains in remission of both diseases. Immune thrombocytopenia is rarely associated with HD and its remission following resection of an accessory spleen is an unusual finding.
Subject(s)
Autoimmune Diseases/diagnosis , Hodgkin Disease/diagnosis , Splenosis/diagnosis , Thrombocytopenia/diagnosis , Adult , Autoimmune Diseases/therapy , Combined Modality Therapy , Hodgkin Disease/therapy , Humans , Male , Radionuclide Imaging , Remission Induction , Spleen/diagnostic imaging , Splenosis/therapy , Technetium , Thrombocytopenia/therapy , Tomography, X-Ray ComputedABSTRACT
Efficacy in the diagnosis of hereditary spherocytosis (HS) using a system of dual-angle differential light scattering of a monochromatic laser beam on sphere-shaped and fixed erythrocytes was evaluated. Fifty-one nonsplenectomized patients with HS, 6 with autoimmune hemolytic anemia, and 140 control subjects were studied. The percentage of erythrocytes with hemoglobin concentration more than 410 g/L (hyperhemoglobin) was significantly different in patients with HS and control subjects (P < 0.001). Hyperhemoglobin could be detected even in the mildest HS, in all patients with autoimmune hemolytic anemia, and in some control subjects, although none of the latter had more than 3%. This technique is extremely sensitive to the presence of spherocytes and the absence of erythrocytes with more than 410 g/L hemoglobin excludes HS. The presence of hyperhemoglobin is not specific for HS and increased amounts of hyperhemoglobin were found in patients with autoimmune hemolytic anemia and control subjects. To make a confident diagnosis of HS, the detection of hyperhemoglobin must be evaluated in the same clinical setting and with the same criteria as those applied to the osmotic fragility test.
Subject(s)
Lasers , Spherocytosis, Hereditary/diagnosis , Erythrocytes/pathology , Hemoglobins/analysis , Humans , Scattering, Radiation , Spherocytosis, Hereditary/bloodSubject(s)
Thrombocythemia, Essential/complications , Urticaria Pigmentosa/complications , Aged , Female , HumansABSTRACT
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.
Subject(s)
Erythrocyte Indices , Erythrocytes, Abnormal/pathology , Flow Cytometry/instrumentation , Hemoglobinometry/instrumentation , Rheology , Spherocytes/pathology , Spherocytosis, Hereditary/pathology , Evaluation Studies as Topic , Humans , Spherocytosis, Hereditary/surgery , SplenectomyABSTRACT
Granulomas in bone marrow are an infrequent finding related to diverse disease. We reviewed 8057 bone marrow studies made over a period of 10.5 years, confirming the presence of granulomas in 40 patients. Global incidence was 0.50% and annual incidence 3.80 cases/yr. Because of the non-specificity of the morphological data, the diagnostic significance of the finding is limited, but it does serve to narrow the field of etiological possibilities. Associated disease was demonstrated in 82.5%, infectious diseases being the most common (tuberculosis, brucellosis, typhoid fever and kala-azar). Two previously unpublished entities are introduced: refractory anemia with excess blast cells (dysmyelopoietic syndrome) and malignant histiocytosis. 3 patients presented human immunodeficiency virus infection, the etiopathogenic role of this retrovirus in the generation of granulomas being unknown. The efficacy of bone marrow study in demonstrating granulomas increases if both the aspirate clot and bone cylinder are examined.
