Long-lasting fever of unknown origin preceding the diagnosis of intravascular lymphomatosis: a further case stimulates some remarks

Neurological and skin involvements usually dominate the clinical presentation of intravascular lymphomatosis (IL), while fever is the most frequent general sign. However, an onset only characterized by fever of unknown origin (FUO) has been rarely reported. We would like to describe a further case of IL, which presented a long-lasting FUO before the diagnosis. At admission, physical examination detected hepatosplenomegaly without lymph nodes enlargement or dermatological or neurological abnormalities. Significant laboratory data included severe anemia, leukopenia, thrombocytopenia, and increased serum LDH. Moreover, a chest CT evidenced bilateral multiple pulmonary infiltrates and pleural effusion. After the development of proteinuria, a diagnosis of large B-cell intravascular lymphoma was made with a renal biopsy 10 months after the onset of the clinical manifestations. So far, more than 100 cases of IL have been reported and the diagnosis often turned out to be difficult, as clinical signs did not point to a lymphoproliferative disorder. This report confirms that FUO is not only frequently associated with IL but that it even marks the real onset of the disease. We are then tempted to conclude that undiagnosed fever is not so rare in IL and if we call it FUO, it is only because diagnosis is necessarily elusive and hence time-consuming. Am. J. Hematol

Follicular spicules and multiple ulcers: cutaneous manifestations of multiple myeloma

We describe 2 patients with multiple myeloma who had horn-like filiform spicules in the follicular orifices of the face, particularly on the nowe, and multiple small ulcerations on the trunk. In the first patient, histopathologic study of a specimen from the nose showed follicular plugs of compact homogeneous eosinophilic material filling the intercellular spaces surrounding the keratinocytes. The same eosinophilic deposits were seen in the ulcer. In the second patient, biochemical investigation revealed that skin matter from spicules and ulcers were made up of monoclonal dysprotein with electrophoretic characteristics identical to those found in patient serum

Cytogenetic and hematological spontaneous remission in a case of acute myelogenous leukemia

Several cases of spontaneous remission (SR) interrupting the invariably progressive course of untreated acute myeloblastic leukemia (AML) have been reported so far. We shall add to this series the hematological and cytogenetic SR occurring in a 72-yr-old man affected by AML following myelodysplastic syndrome. At diagnosis cytogenetic analysis showed the 48, xy, del (6) (p22-pter), +13, +14 karyotype. Owing to a lobar pneumonia, the chemotherapy was deferred and a broad spectrum antibiotic therapy was established. Supportive care included red cells and platelet transfusions and low-dose corticosteroid. Two months later, after the pneumonia had completely disappeared, a complete remission, lasting about 5 months, was documented on bone marrow morphological and cytogenetical examination, although some degree of myeloid dysplasia persisted. Possible mechanisms of the various SRs described during the course of AML are discussed with a review of the literature

Maximal γ-globin expression in the compound heterozygous state for –175 ^Gγ HPFH and β⁰39 nonsense thalassaemia: a case study

The –175 (T→C) Gγ hereditary persistence of fetal haemoglobin is a very rare promoter mutation occurring in Caucasians as well as in African-Americans. Heterozygotes for this non-deletional HPFH show 20% HbF, mostly of Gγ type. We describe here a healthy Sardinian man who coinherited –175 (T→C) Gγ HPFH with the β-thalassaemia codon 39 nonsense mutation in trans; he showed 64% HbF, 100% of Gγ type. Although the β-globin haplotype pattern (II/II) was indicative of the presence of the AγT allele on both chromosomes, the AγT expression was undetectable by HPLC even in red cell populations separated by age. The proband was, moreover, homozygous for the –4 bp deletion at position -225 to -222 of Aγ promoter which has recently been associated with decreased AγT globin expression. These findings suggest that this maximal overexpression of Gγ-globin probably reflects intensified stimulation of the mutated Gγ promoter in this hitherto undescribed genetic condition

Strabismus and diplopia in a patient with acute myeloid leukemia

Background: Central nervous system (CNS) involvement is a sporadic presenting finding in patients with acute myeloid leukemia (AML) both at diagnosis and at relapse. Moreover patients with CNS localization are often asymptomatic, while sometimes show meningeal signs and symptoms or, extremely rarely, signs of cranial nerve impairment. Case Report: Here we report on a patient with refractory AML who suddenly developed strabismus and diplopia. Both neurological and ophtalmologic examinations were suggestive of a bilateral VI cranial nerve palsy. Noteworthy, both a cranial CT and MRI were substantially normal, while a rachicentesis was performed and cerebrospinal fluid examination was clearly suggestive of a meningeal involvement by AML. Conclusions: This is to our knowledge the first reported case in which the clinical picture of meningeal localization in an AML patient was dominated by an isolated abducens cranial nerve impairment. Moreover it highlights as unexplained strabismus and diplopia can be considered as a potential sign of CNS involvement, even if conventional imaging is negative

Glucose-6-phosphate dehydrogenase deficiency and risk of invasive fungal disease in patients with acute myeloid leukemia

Invasive fungal diseases (IFD) are still a leading cause of morbidity and mortality in patients with acute myeloid leukemia (AML). Glucose-6-phosphate dehydrogenase is an enzyme that leads to the production of NADPH, required to destroy microorganisms in the respiratory burst reaction of white blood cells. We evaluated the role of G6PD deficiency in susceptibility of IFD in 108 AML patients undergoing intensive chemotherapy. In all, 28 patients harbored G6PD deficiency (G6PD-), whereas 80 were normal (G6PD +). Incidence of IFD was significantly higher in G6PD- patients compared to G6PD + patients (35.7% vs. 5%, p = .0002, OR = 10, 95% CI = 2.96-37.5). Higher risk of mold infections (17.9% vs. 5%, p = .048, OR = 4.1, 95% CI = 1.0-16.6) and Candida sepsis (17.9% vs. 0%, p = .0009, OR = 37.68, 95% CI =2.0-707.1) was observed in G6PD - patients. The evaluation of G6PD activity may help to identify AML patients at higher risk of IFD, allowing to design more intensive surveillance and therapeutic strategies

Male Predominance of Gastric Cancer among Patients with Hypothyroidism from a Defined Geographic Area

In the past, hypothyroidism has been associated with an increased susceptibility to gastric cancer (GC). Although several epidemiological studies have corroborated this association, a precise mechanistic explanation remains elusive. In this study, this hypothesis was tested by using a large database of subjects who underwent upper endoscopy for various reasons. This was a retrospective, case-control, single-center study. Subjects with GC (cases) were compared with subjects without (controls), according to hypothyroidism status. Overall, the prevalence of GC was 0.73% in the total cohort and was significantly higher in males compared to females (1.4% versus 0.4%, p < 0.0001). Multivariate logistic regression analysis confirmed an increased risk in males with hypothyroidism (OR 5.10; p < 0.0001) after adjusting for potential confounders, especially H. pylori infection. Interestingly, only hypothyroidism and not treatment with levothyroxine was a significant predictor of GC, ruling out a possible direct carcinogenic effect of the replacement therapy. The present study suggests a male-restricted association of gastric carcinogenesis with a hypothyroid state. If the results of this study are confirmed by longitudinal studies, an attractive perspective could open up for the better management of males with concomitant hypothyroidism and a higher risk of GC

Epidemiological updates and economic losses due to <i>Taenia hydatigena</i> in sheep from Sardinia, Italy

The aim of this study was to investigate the epidemiology and transmission of Taenia hydatigena in sheep and dogs from Sardinia and the economic estimation of losses due to this metacestodosis in lambs. A total of 7781 Sarda breed lambs were examined at abattoirs for the detection of Cysticercus tenuicollis or necrotic–haemorrhagic tracks of their migration. Morphological and molecular identification of parasites was carried out. Individual faecal samples from 300 dogs were examined for copromicroscopic investigations and coproELISA assay. An overall prevalence of 14.6 % for T. hydatigena cysticercosis was found in the examined lambs. In total, 10,807 parasitary tracks were found, with an abundance of 1.39 and an average intensity of 9.52. The molecular analysis of the isolates showed an overall pairwise nucleotide divergence for the CO1 and ND1 was of 0–3.1 and 0–3.3 %, respectively. Low intra- and interspecific variation was recorded for C. tenuicollis isolates used in this study which suggested the absence of differentiation. Microscopic examination of dog faeces showed a total prevalence of 31.3 % for endoparasites in the examined samples (94/300). Taeniid eggs were found in 8.3 % of the dogs. The results of the monoclonal antibody ATH4 ELISA test showed a prevalence of 11 % (33/300) for T. hydatigena coproantigens. The total economic costs related to cysticercosis amounted to almost € 330,000. The prevalence of C. tenuicollis in 14.6 % of 30–40-day-old lambs highlights the high parasitic pressure by T. hydatigena in the territory of Sardinia, Italy

Hematological and biosynthetic features of β⁰-thalassemia with an associated α-thalassemia heterozygosity in Sardinia

Total hemoglobin, PCV, hemoglobin A2 and hemoglobin F were determined in controls, β0-thal heterozygotes and α-thal+ β thal heterozygotes. Also the distribution of mean corpuscular volume and the mean corpuscular hemoglobin values in Sardinian β0-thal heterozygotes with and without an associated α-thal and the ratio of α/β chain synthesis in β0-thal heterozygotes with or without an associated α-thal were studied