Acta haematologica
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At onset mitochondrial disorders (MID) frequently manifest as a mono-organic problem but turn into multisystem disease during the disease course in most of the cases. Organs/tissues most frequently affected in MID are the cerebrum, peripheral nerves, and the skeletal muscle. Additionally, most of the inner organs may be affected alone or in combination. ⋯ In single cases with Leigh's syndrome, MERRF (myoclonic epilepsy and ragged-red fiber) syndrome, Leber's hereditary optic neuropathy, and Friedreich's ataxia anemia has been described. Anemia, leukopenia, thrombocytopenia, eosinophilia, or pancytopenia can frequently also be found in nonsyndromic MIDs with or without involvement of other tissues. Therapy of blood cell involvement in MID comprises application of antioxidants, vitamins, iron, bone marrow-stimulating factors, or substitution of cells.
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Case Reports
Two cases of ampulla (takotsubo-shaped) cardiomyopathy associated with hemophagocytic lymphohistiocytosis.
There have been many reports of patients with ampulla cardiomyopathy described as takotsubo-shaped cardiomyopathy in the cardiovascular field. This unique cardiomyopathy is characterized by transient apical ballooning and hypokinesis of the left ventricle. We describe 2 cases of ampulla cardiomyopathy associated with hemophagocytic lymphohistiocytosis (HLH). ⋯ In each case, the findings on ECG, echocardiogram and left ventriculogram were compatible with ampulla cardiomyopathy. To our knowledge, this communication is the first to report cases of ampulla cardiomyopathy associated with HLH. Our cases suggest that HLH hypercytokinemia may have a role in causing ampulla cardiomyopathy.
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Since gastric acidity and ascorbate play a critical role in the solubilization and reduction of iron for subsequent absorption, the achlorhydria associated with autoimmune and Helicobacter pylori gastritis may explain the poor response of such patients to oral iron treatment. In order to circumvent this problem, we explored the therapeutic potential of a duodenal formulation of ferrous glycine sulfate consisting of micropellets that do not dissolve at the acid environment of the stomach but, owing to their solubility at a higher pH, discharge their content directly into the duodenum. ⋯ In patients with iron deficiency anemia associated with autoimmune and H. pylori gastritis with a high rate of refractoriness to oral iron treatment, satisfactory response to a duodenal formulation of ferrous glycine sulfate can be elicited in the vast majority of cases, obviating the need for expensive, inconvenient and occasionally risky intravenous iron administration.
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Although the presence of decreased plasma fibrinogen has been regarded as an indicator of ongoing disseminated intravascular coagulation (DIC), fibrinogen, which is one of the acute phase reactants, is often increased in the patients with DIC. We investigated the diagnostic and prognostic utility of a new parameter [the fibrinogen/C-reactive protein (CRP) ratio] for predicting DIC in 1,056 patients with suspected DIC and who also had underlying disorders associated with DIC. Among the 535 patients with overt DIC, 46 patients (8.6%) showed low plasma fibrinogen (<100 mg/dl), suggesting that the plasma fibrinogen level is not a sensitive marker for DIC. ⋯ The area under the receiver-operating characteristic curve of the fibrinogen/CRP ratio, when this was used for predicting mortality, showed significantly better discriminative power than did that of the fibrinogen level. This study demonstrates that the fibrinogen/CRP ratio may provide more discriminating power for identifying the patients with active coagulation consumption, and the fibrinogen/CRP ratio has a good predictive value concerning the 28-day mortality in the patients suspected of having DIC. The results of our study suggest that replacement of fibrinogen by the fibrinogen/CRP ratio for calculating the DIC score may lead to enhance diagnostic and prognostic power for DIC.