Aplastic hypoplastic anaemia

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No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

No abstract text is available yet for this article.

Bone marrow failure is believed to be the underlying condition that drives the expansion of the paroxysmal nocturnal haemoglobinuria (PNH) clone. Indeed, circulating PNH blood cells have been identified in patients with acquired aplastic anaemia and with hypoplastic myelodysplasia. Whether PNH blood cells are also present in patients with inherited aplastic anaemia has not been reported. We screened a large group of patients diagnosed with Shwachman-Diamond Syndrome (SDS) for PNH blood cells. None of the patients analysed had detectable circulating PNH blood cells, indicating that bone marrow failure in SDS does not select for PNH progenitor cells...

OBJECTIVE: To determine the relative efficacy of bone marrow aspiration as compared to that of trephine biopsy. METHODS: Bone marrow aspiration and bilateral trephine biopsies were performed in 420 consecutive cases. The diagnosis and findings made on bone marrow aspiration were compared with that made on trephine biopsy in each case. RESULTS: Aspiration alone was sufficient in making a diagnosis in 372 (88.6%) cases as it correlated well with the diagnosis made on trephine sections...

The clinical outcome of childhood aplastic anaemia (AA) with aberrant cytogenetic clones at diagnosis was surveyed. Among 198 children with newly diagnosed AA registered with the AA Committee of the Japanese Society of Paediatric Hematology between 1994 and 1998, cytogenetic studies of bone marrow (BM) cells were completed in 159 patients. Apart from one Robertsonian translocation, seven patients (4.4%) showed clonal chromosomal abnormalities in hypoplastic BM without myelodysplastic features. The patients included six girls and one boy with a median age of 11 years (range 5-14 years)...

It is often difficult to distinguish hypoplastic myelodysplastic syndrome (h-MDS) from acquired aplastic anaemia (AA), because of the considerable clinical, cytological and histological similarities between these two disorders. The distinction between AA and h-MDS is important because there is a higher risk of progression to acute leukaemia in patients with h-MDS compared with AA. Recent studies suggest that tumour necrosis factor-alpha (TNF-alpha) plays an important role in the development of AA. In order to determine the potential importance of TNF-alpha in the differential diagnosis of hypoplastic bone marrow (BM) disorders, we examined whether analysis ofTNF-receptor expression could be used as a tool to differentiate AA from h-MDS...

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by intravascular haemolysis, venous thrombosis, marrow hypoplasia, frequent episodes of infection, and rarely leukaemic conversion. At the cellular level, PNH is characterized by the decrease or absence of glycosylphosphatidylinositol (GPI)-anchored molecules, such as CD55 and CD59, from the cell surface. PNH-like clones have been described in various haematological disorders. The link between PNH and aplastic anaemia (AA) has been established but the relationship of PNH with myelodysplastic syndromes (MDS) or myeloproliferative disorders (MPD) remains unclear...

GnRH agonists, applied for a long period of time or as depot forms, lead to blocking of gonadotropin and ovarian steroid synthesis. We used their property to induce amenorrhea for treating menorrhagic and menometrorrhagic bleeding in adolescence caused by inborn aplastic and hypoplastic anaemia. In patients with uterine bleeding during the first regular menstrual period or recurring during menstrual periods Zoladex (depot GnRH agonist) has been applied, resulting in stop of bleeding in 2 to 4 days. The drug-induced amenorrhea provides the possibility for physiologic restoring of hemoglobin levels, improvement of therapeutic results from adjuvant antianaemic therapy...

AIM OF STUDY: To study the prevalence, clinico-haematological and management profile of aplastic anaemia (AA) among severely anaemic patients treated at BP Koirala Institute of Health Sciences (BPKIHS), Dharan, Nepal. METHOD: A retrospective analysis of 18 cases of biopsy proven AA patients was done between September 1995 and August 1997. RESULTS: Over a period of 2 years, 140 patients with severe anaemia were admitted to our institution. Eighteen patients were diagnosed with AA...

Recently the number of long-term survivors of aplastic anaemia has increased, with some of these cases evolving into myelodysplastic syndrome (MDS). Because it is difficult to discriminate between aplastic anaemia and hypoplastic MDS, it is unknown whether these patients have had MDS from the time of diagnosis of aplastic anaemia. Presence of ringed sideroblasts on an iron-stained bone marrow smear is a characteristic of some cases of MDS. Amorphous electron-dense deposits in the mitochondrial matrices of erythroid precursors observed with an electron microscope show ringed sideroblasts, and detection of this mitochondrial pathology is useful for confirming the presence of ringed sideroblasts because this mitochondrial pathology can be found not only in erythroblasts but also in reticulocytes, which is particularly useful in cases in which few erythroblasts are found...

The case of a 68 year old women presenting in myxoedema coma is described. She was found to be anaemic with a haemoglobin of 8.2 g/dl. Further investigations showed a pancytopenia and a hypoplastic anaemia confirmed by bone marrow. The patient recovered and became euthyroid following initial treatment with intravenous tri-iodothyronine (T3) and later oral thyroxine (T4) replacement with resolution of pancytopenia and return of bone marrow to normal.

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder of haematopoiesis. Clinically it is characterized by intravascular haemolysis, venous thrombosis and often by bone marrow hypoplasia. Haemolysis and thrombosis develop as a consequence of deficiency of several proteins on the cell membrane of the affected clone of blood elements. This is caused by somatic mutations in the PIG-A gene, which encodes an enzyme involved in the biosynthesis of glycosylphosphatidylinositol (GPI) anchor. Spectrum of mutations in the PIG-A gene is different to that observed in other genes...

Iron overload is a serious complication of many forms of anaemia, arising in part from mechanisms associated with compensatory increases in erythropoiesis. To investigate other mechanisms by which anaemia itself may perturb iron metabolism, without the confounding effects of compensatory erythropoiesis, we studied transgenic mice with a partially disabling insertion in the erythropoietin gene, which manifested as incomplete erythropoietin deficiency. Mice were studied aged 7-8 weeks. Haemoglobin concentrations were 6...

Clinical evidence for a link between aplastic anaemia, paroxysmal nocturnal haemoglobinuria (PNH) and hypoplastic leukaemia is provided by studies of clonal disorders, which may be a complication of congenital or acquired aplastic anaemia. Fanconi's anaemia is the most common congenital disorder and leukaemia occurs in at least 10% of cases. In acquired aplastic anaemia, a high incidence of myelodysplastic syndrome (MDS) was noted in patients with aplastic anaemia, seemingly cured of their aplasia by antilymphocyte globulins (ALG)...