Copper levels in patients with hematological malignancies.

BACKGROUND: Copper levels are elevated in cancer patients compared to normal subjects. However, few studies have investigated the relationship between copper and hematological malignancies. METHODS: 84 patients with hematological diseases were studied, along with 50 healthy individuals. Copper was measured by flame atomic absorption spectrometry. The patients were classified to 2 homogeneous groups, acute and chronic hematological neoplasms, respectively. For the patients with acute hematological malignancies, relapse and remission were investigated in relation to serum copper levels. For chronic hematological neoplasms, serum copper was connected either with stable or progressive disease. Zeta-chain-associated protein kinase 70 (ZAP70) and CD38 expression, along with the unmutated VH immunoglobulin genes (IgVH) status were also determined for the 22 chronic lymphocytic leukemia (CLL) patients. RESULTS: 54 patients with relapse or progressive disease had elevated copper levels (mean value 1.8 mg/l), whereas 30 patients either in remission or in stable disease had normal copper levels (mean value 1.01 mg/l) (normal range 0.8-1.3mg/l). CONCLUSION: Hence, our study indicates that serum elevated copper levels are associated with hematological malignancies either in relapse or in disease progression, whereas normal copper levels are linked with hematological neoplasms in remission or in stable disease. Furthermore, we report for the first time an association between high serum copper levels and several adverse prognostic markers in CLL, such as increased expression of ZAP70 and CD38, along with elevated percentage of unmutated IgVH.

Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task.

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) is a rare and distinct neoplasm appearing de novo on the skin. We present a case of a 75-year-old man diagnosed with PC-ALCL in his left femoral region. We describe the morphology of lesions along with the differential diagnosis, treatment, clinical course and prognosis. We further discuss parameters concerning treatment that should be considered when a PC-ALCL is diagnosed. Our case report demonstrates the complexity in classification, staging, differential diagnosis and therapy selection of PC-ALCLs. It is crucial to emphasize the importance of clinical criteria in diagnosing a PC-ALCL in combination with immunohistochemistry.

Increased incidence of iron deficiency anemia secondary to inadequate iron intake in institutionalized, young patients with cerebral palsy.

We observed high incidence of anemia in patients with cerebral palsy sheltered in a specialized institution in Thessaloniki, Greece. Therefore, we decided to investigate its cause. We studied 108 patients, and assessed complete blood cell count, peripheral blood smear, serum iron, ferritin, folate, B12 and the presence of hemoglobin or parasites in the stools. In all cases, anemia was hypochromic and microcytic. Approximately 33% of patients suffered from hypochromic anemia, whereas 38% were iron deficient. There was no statistical difference in the incidence of iron deficiency between different age groups. All tests for fecal occult blood or intestinal parasites were negative. Folic acid and B12 levels were within normal range in all cases. We also found that 87 and 95.6% of patients on liquid diet were anemic and iron deficient, respectively, compared to only 18.8 and 22.3% of patients on normal diet. The high incidence of anemia was attributed to iron deficiency which was secondary to inadequate iron intake and decreased iron absorption. Thus, it would not be irrational to consider iron supplementation as the first measure in such patients and postpone endoscopic procedures for a later stage, unless there are clinical or laboratory findings (such as fecal occult blood) suggestive of gastrointestinal blood loss.

Veno-occlusive disease of the liver during induction therapy for acute lymphoblastic leukemia.

Veno-occlusive disease (VOD) of the liver, also termed as sinusoidal obstruction syndrome, constitutes a well-known complication of high-dose cytoreductive chemotherapy prior to allogeneic or autologous bone marrow transplantation and is associated with the intensity of treatment [1]. On the contrary, there is only one report of VOD during induction therapy for acute lymphoblastic leukemia in a patient with Marfan syndrome, who was successfully treated with defibrotide [2]. In this article, we present the first fatal case of VOD during induction therapy for acute lymphoblastic leukemia.

Increased values of mean platelet volume and platelet size deviation width may provide a safe positive diagnosis of idiopathic thrombocytopenic purpura.

INTRODUCTION: It has been shown recently that platelet indices like mean platelet volume (MPV), platelet size deviation width (PDW) and platelet-to-large-cell ratio (P-LCR) are helpful in the discrimination between hyperdestructive thrombocytopenia like idiopathic thrombocytopenic purpura (ITP) and hypoproductive thrombocytopenia (HT). The aim of the study is to assess the reliability of these indices in the differentiation of ITP from other thrombocytopenias. METHODS: We recruited 134 thrombocytopenic patients (69 men, 65 women) who were divided into two groups according to the underlying disease: group I (n = 63) included ITP patients, whereas group II (n = 71) included patients with HT due to myelosuppression secondary to chemotherapy for hematological malignancies. Platelet indices were derived from a Sysmex automated cell counter. Sensitivity, specificity, positive prognostic value, negative prognostic value, efficiency and Youden index were calculated. RESULTS: Concerning MPV and PDW indices, sensitivity, specificity, positive prognostic value, negative prognostic value, efficiency and Youden index were 100% for the diagnosis of ITP. On the contrary, the values for P-LCR were significantly lower. CONCLUSIONS: MPV and PDW can be safely relied on for a positive diagnosis of ITP. MPV and PDW were superior to P-LCR.

Detection of CD55- and CD59-deficient granulocytic populations in patients with myelodysplastic syndrome.

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by absence of CD55 and CD59 from the surface of affected cells. PNH has been associated with myelodysplastic syndromes (MDS). The aim of our study was to estimate the prevalence of the PNH clone in MDS patients by detecting CD55 and CD59 deficiency. We studied 90 MDS patients: 19 patients with RA, 15 with refractory anemia with ringed sideroblasts (RARS), 18 with refractory anemia with excess of blasts (RAEB), 17 with refractory anemia with excess of blasts in transformation (RAEB-t), and 21 with chronic myelomonocytic leukemia (CMML). Twenty healthy individuals were also studied as the control group. We studied the PNH clone on granulocytes of these patients with the aid of flow cytometry. CD55- and CD59-deficient granulocytic populations were detected in 15.5% of MDS patients compared to 2.8% of normal individuals. Among the subgroups of the study, significant difference was present in three cases: (1) between CMML and control, (2) between CMML and RA, and (3) between CMML and RARS. These data indicate a possible association between PNH phenotype and MDS. MDS patients of worse prognosis (CMML) express more strongly the PNH clone compared to those of better prognosis (RA and RARS). Perhaps, the examination of MDS patients for the PNH clone by flow cytometry could provide us with a valuable prognostic tool.

Discrimination indices as screening tests for beta-thalassemic trait.

The two most frequent microcytic anemias are beta-thalassemic trait (beta-TT) and iron deficiency anemia (IDA). Several discrimination indices have been proposed to distinguish between these two conditions. These indices are derived from several simple red blood cell indices, like red blood cell (RBC) count, mean cell volume, and RBC distribution width (RDW), as these are provided by electronic cell counters. The purpose of the study is to examine the diagnostic accuracy of six discrimination indices in the differentiation between IDA and beta-TT. The six discrimination indices that were examined were as follows: Mentzer Index (MI), Green & King Index (G&K), RDW Index (RDWI), England & Fraser Index (E&F), RDW, and RBC count. We calculated these indices on 373 patients (205 men, 168 women) with beta-TT and 120 patients (50 men, 70 women) with IDA, as well as their sensitivity, specificity, positive and negative prognostic value, efficiency, and Youden's index (YI). G&K shows the highest reliability, followed by E&F, RBC count, MI, and RDWI. On the contrary, RDW completely failed to differentiate between IDA and beta-TT. G&K proved to be the most reliable index as it had the highest sensitivity (75.06%), efficiency (80.12%), and YI (70.86%) for the detection of beta-TT. These six discrimination indices cannot be relied on for a safe differential diagnosis between beta-TT and IDA. They do have high specificity, but their sensitivity for the detection of beta-TT is not satisfactory. Consequently, they cannot be used neither as a screening tool for beta-TT because they could result in a significant number of false negative results.