Cases reported "Hematologic Diseases"

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1/28. Importance of basophilia in haematopoietic disorders.

    To the significance of basophilia in haematopoietic disorders, six draw attention to cases have been analyzed. Associated diseases included acute myelogenous leukaemia (AML-M2, M3, M4, and M6), refractory anaemia with excess of blasts (RAEB) and RAEB in transformation (RAEB-T). Two AML cases (M2, M6) were preceeded by myelodysplastic syndromes (MDS). All patients showed greater than 3% basophilia in peripheral blood and bone marrow. basophils were identified successfully by metachromatic staining with toluidine blue in all cases. Three patients (M3, M4, RAEB) presented with lymphadenopathy, suggesting an association with extramedullary involvement. Neutrophil alkaline phosphatase (NAP) activity was significantly reduced in four patients with AML (M2, M3, M4) and RAEB-T. The clinical course was generally unfavourable characterized by short remission duration or disease progression except for the patient with RAEB. Haemorrhage was the main cause of death rather than infection. cytogenetic analysis revealed unique abnormalities involving chromosomes 3q21, 5q31, and 17q11 where the genes for some haematopoietic growth factors or their receptors are located, in addition to t(6;9) and t(15;17).
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2/28. The diagnosis from the pathological viewpoint of a blood disease.

    The histopathological viewpoints of a blood diseases are namely following the cytological examinations except for the viewpoint of organ pathology and has been required only the usefulness in case with the difficulty of cytological examination. If it looks at a blood disease as hematopoietic organ disease, histopathological diagnostic study might make the new paradigm of blood diseases. In this time, I would like to present the hematological diagnosis from the pathological viewpoint by using histological bone marrow sections. *How to examine the bone marrow histology *Hematopoietic microenvironment disorder *Differential diagnosis of hypoplastic marrow lesions *Clinicopathological characteristics of Hypoplastic leukemia *Hemophagocytosis in bone marrow *How to diagnose MDS by histopathology. Bone marrow histology is the valuable diagnostic tool of many kinds of marrow disorders especial cases. By using immunohistochemistry and Giemsa staining, further information might obtain than smear film cytology. Bone marrow clot section aspirated from sternum is enough for histological examination except bone marrow biopsy. Precise cytomorphology might demonstrate by smear film than section histology. However surface phenotype would define by flow cytometric analysis, immunostaining of sections could demonstrate which cells show which markers. Structural and architectural disorder could only be represent by histology. The histological examination of bone marrow might introduce a new aspects of blood disease.
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3/28. Systemic polyclonal B-immunoblastic proliferation with marked peripheral blood and bone marrow plasmacytosis.

    The clinical and pathologic features of a case of acute systemic polyclonal B-immunoblastic proliferation characterized by pronounced peripheral blood and bone marrow plasmacytosis and infiltration of the hepatic portal areas by immunoblasts, plasma cells, and lymphocytes are reported. Clinical and laboratory findings during the acute phase and long-term follow-up support the diagnosis of a benign process, possibly related to pseudomonas aeruginosa septicemia. The patient experienced a dramatic clinical recovery on administration of high-dose intravenous corticosteroids. Pathologists should be aware of this entity so as not to confuse it with non-Hodgkin's lymphoma or a form of plasma cell dyscrasia.
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4/28. Unusual haematological alterations in rheumatoid arthritis.

    Three cases of rheumatoid arthritis (RA), presenting with refractory anaemia, thrombocytopenia and peripheral lymphocytosis respectively, were observed. In all the cases haematological manifestations were unrelated to disease activity or drug toxicity. These patients were detected to have pure red cell aplasia (PRCA) (normocytic normochromic anaemia, reticulocytopenia and absence of erythroid precursors in the bone marrow), immune thrombocytopenia (IT) (absence of splenomegaly and presence of increased megakaryocytes in the bone marrow) and multiple myeloma (MM) (lytic lesions on skull, paraproteinaemia and bone marrow plasmacytosis) respectively. PRCA and IT responded to glucocorticoids. association with these three haematological alterations has rarely been reported. Our report highlights the need to regularly monitor blood counts in patients with RA.
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5/28. A case of lichen myxedematosus associated with immunological abnormalities.

    A 33-year-old male consulted us with symptoms of band-like infiltration, thickenning and subcutaneous induration occurring in the face, chest, back, shoulder and upper extremities. In the following time he developed arthralgia, fever and ocular symptoms. Laboratory tests revealed normal thyroid functions and a normal bone marrow picture, but there were immunological abnormalities such as reduced cellular immunity, decrease in C 3 (beta 1c/la) and positive antinuclear antibody. Histologically, there was an increase in fibroblasts and deposits of hyaluronic acid were revealed histochemically. A diagnosis of lichen myxedematosus was established. At present, all symptoms and signs including skin rash have disappeared following corticosteroid therapy and the patient is being followed up. So far as we have been able to trace, reports on lichen myxedematosus associated with immunological abnormalities as described here have not been found. The relationship between this pathology and immunological abnormalities are discussed.
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6/28. helicobacter pylori as cause of gastrointestinal disease in children with hemato-oncologic diseases.

    After documentation of a case of life threatening helicobacter pylori (H. pylori) gastric ulcer in an adolescent girl on treatment for acute lymphoblastic leukaemia, we started to systematically look for gastro-intestinal symptoms due to H. pylori infection in our cancer patients at G. Gaslini Children's Hospital. During a period of 46 months, we observed 13 further cases of severe dyspepsia syndrome or gastro intestinal bleeding associated with presence of H. pylori faecal antigen. All patients recovered with appropriate therapy. H. pylori may represent a cause of severe gastrointestinal complications in children with cancer or following bone marrow transplant.
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7/28. Limited rescue of osteoclast-poor osteopetrosis after successful engraftment by cord blood from an unrelated donor.

    We report on a case of osteoclast-poor osteopetrosis who received a hematopoietic stem cell graft and, despite hematological engraftment, showed little signs of response in the skeletal defect. Clinical and laboratory studies supported the concept that the bone microenvironment remained abnormal, thus reducing the clinical response to transplantation. INTRODUCTION: osteopetrosis is a rare genetic disorder characterized by severely reduced bone resorption resulting from a defect in either osteoclast development (osteoclast-poor osteopetrosis) or activation (osteoclast-rich osteopetrosis). patients with osteoclast-rich osteopetrosis can be rescued by allogenic hematopoietic stem cell transplantation; however, little information exists concerning the success of transplantation as a treatment for osteoclast-poor osteopetrosis. We report on a child with osteoclast-poor osteopetrosis whose diagnosis was delayed, consequently receiving a cord blood transplant from an unrelated donor at the age of 8 years. Engraftment was deemed successful by peripheral blood genotyping, although >3 years after transplantation there was little rescue of the skeletal defect and anemia, and extramedullary hematopoiesis persisted. MATERIALS AND methods: Peripheral blood mononuclear cells from the osteopetrosis patient, before and after transplantation, were used to generate osteoclasts in vitro in the presence of macrophage colony-stimulating factor (M-CSF) and RANKL. RESULTS: Before transplantation few, small mononuclear osteoclasts formed (F-actin ring-positive cells, co-localizing with vitronectin receptor [alphavbeta3 integrin] and TRACP) associated with occasional, small resorption lacunae. Low levels of collagen C-terminal telopeptide (CTx) fragments were released from these cultures as assessed by ELISA (CrossLaps; patient, 12.85 nM; control, 448.6 nM). In contrast, osteoclasts formed in cultures after transplantation formed to a similar degree to control cultures from healthy individuals: large numbers of osteoclasts containing numerous nuclei were present, and approximately 50% of the surface of bone slices was resorbed, associated with intermediate levels of collagen fragment release (116.48 nM). The culture data reflect the histopathology and radiological findings and also support previous studies showing that neither M-CSF nor RANKL rescues osteoclast-poor osteopetrosis. CONCLUSIONS: This is the first case reported in which a successful hematopoietic engraftment failed to correct an osteopetrotic skeletal defect, and this finding may be credited to the age at which the child was transplanted.
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keywords = macrophage, bone
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8/28. Interstitial deletion of chromosome 5, del(5q), in a newborn with down syndrome and an unusual hematologic disorder.

    A newborn with down syndrome was noted on the 1st day of life to have an elevated white blood cell count of 79,900/mm3 with 62% lymphoblasts and a platelet count of 61,000/mm3, consistent with either transient myeloproliferative disorder of down syndrome (TMD) or acute leukemia. karyotype analysis of a bone marrow aspirate revealed that 20% of the cells had a 47,XY, 21 karyotype, and 80% had a 47,XY, 21, del(5)(q13q31) complement. Cytochemical and immunophenotyping of the peripheral blasts were consistent with the presence of an acute undifferentiated precursor blast clone. Results of clonogenic assays of hematopoietic progenitors from this patient's bone marrow were similar to those of patients with TMD. This patient's hematologic abnormalities resolved spontaneously without treatment by week 10 of life. This is the first report of an interstitial deletion of 5q associated with a hematologic abnormality present in an infant at birth.
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9/28. New clinical aspects of Pearson's syndrome. Report of three cases.

    We describe 3 patients affected by Pearson's syndrome, presenting anemia, exocrine pancreas failure, and skeletal abnormalities; insulin-dependent diabetes mellitus arose in two cases during the course of the disease. Bone marrow dysplasia and exocrine pancreas failure are also reported in Shwachman's syndrome; the two forms differ in bone marrow morphology. The clinical pattern of Pearson's syndrome can be so polymorphic as to increase the difficulties of differential diagnosis with Shwachman's syndrome.
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keywords = bone
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10/28. Successful HLA nonidentical bone marrow transplantation in three patients with the leukocyte adhesion deficiency.

    Three consecutive patients with the severe phenotype of leukocyte adhesion deficiency characterized by a defective expression of LFA-1, Mac-1 (CR3), and p150.95 on leukocytes have received HLA partially incompatible bone marrow transplantation (BMT). The degree of HLA incompatibility between related donors and recipients was 2 hla antigens in one and one full haplotype in the two others. Graft-v-host disease (GVHD) prophylaxis consisted in T-cell depletion of the bone marrow inoculum and a 60-day course of cyclosporin A. A first attempt led to autologous recovery in one patient. The second transplant in this patient and the first transplant in the two others led to stable partial engraftment of lymphocytes and phagocytic cells, as shown by expression of adhesion molecules (LFA-1, Mac-1) on leukocytes and by HLA typing and restriction fragment-length polymorphism studies using minisatellite probes. Although the level of mixed chimerism was lower in one patient (7% to 30% donor cells) and greater than 50% in the two others, recovery of lymphocyte and phagocytic cell functions was sufficient enough to allow the patient to lead a normal life, infection free in the three cases. These patients, now 57, 32, and 19 months post-transplant, are in good condition without any therapy. These results lead us to propose that the LFA-1 molecule plays a role in HLA-incompatible graft rejection, probably by mediating adhesion of cytotoxic T and non-T lymphocytes to their targets.
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