Nese Yarali

Most reliable indices in differentiation between thalassemia trait and iron deficiency anemia

Background : Iron deficiency anemia (IDA) and thalassemia trait (TT) are the most common forms of microcytic anemia. Some discrimination indices calculated from red blood cell indices are defined and used for rapid discrimination between TT and IDA. However, there has been no study carried out in which the validity of all of the defined indices are compared in the same patient groups. Youdens index is the most reliable method by which to measure the validity of a particular technique, because it takes into account both sensitivity and specificity.

Hematologic Manifestation of Childhood Celiac Disease

We wanted to describe the hematologic manifestations of celiac disease (CD) in childhood. This study included 22 children with CD in whom the disease remained undiagnosed until they had presented with hematological abnormalities, such as anemia, thrombocytopenia, leukopenia or prolonged prothrombin time (PT) and activated partial thromboplastin time (APTT). Anemia was present alone in 19 (86.3%) patients, and leukopenia coexisted with anemia in 2 (9%) patients. Thrombocytopenia was found alone in 1 (4.5%) patient. Twelve patients had an iron deficiency anemia. Iron deficiency coexisted with zinc and vitamin B12 deficiency in 3 patients, copper and vitamin B12 deficiency in two, vitamin B12 deficiency in two, zinc deficiency in two and one patient had combined iron, zinc, and copper deficiency. Males had significantly lower values of hemoglobin (p < 0.05) and MCV (p < 0.05) compared to the females. In conclusion CD should be included in the differential diagnosis in children who present with anemia, leukopenia, thrombocytopenia or prolonged PT and APTT, especially in geographical areas where the prevalence of the CD is high.

CLINICAL COURSE OF CHILDREN WITH IMMUNE THROMBOCYTOPENIC PURPURA TREATED WITH INTRAVENOUS IMMUNOGLOBULIN G OR MEGADOSE METHYLPREDNISOLONE OR OBSERVED WITHOUT THERAPY

The authors compared the prognosis in 50 children with acute immune thrombocytopenic purpura (ITP) who received intravenous immunoglobulin G (IVIG), megadose methylprednisolone (MDMP), or no therapy. Twenty-six children were observed with no therapy, 12 children received IVIG, and 12 children received MDMP. The percentage of the patients whose platelet counts increased at a level of > 20 2 10 9 /L and > 50 2 10 9 /L at 3 days after starting therapy was significantly higher in both IVIG and MDMP groups than in the no therapy group ( p < .01), but there was no significant difference at 10 and 30 days after initiation between the 3 groups ( p > .05 in each comparison). This result suggested that therapy does not increase the rate of recovery but shortens the duration of thrombocytopenia in the first days. Management decision in ITP is made on clinical condition rather than on platelet count and no treatment options is to be preferred even in the face of mucosal bleeding. If the patient has extensive bleeding and the decision is to treat, both IVIG and MDMP are equally effective in providing a safe platelet level early on.

Neonatal thrombo-embolism: risk factors, clinical features and outcome

Abstract Background: There are few data with respect to prothrombotic risk factors in neonates. Aim: To determine the associated risk factors, clinical features and outcome in newborn infants diagnosed with thrombo-embolism. Methods: Case records of 25 infants (17 full-term and eight preterm) diagnosed with thrombo-embolism between January 2005 and April 2008 in a neonatal intensive care unit were reviewed. Results: Of the 25 infants, 18 cases of venous (72%) and seven of arterial (28%) thrombo-embolism were recorded; in 18 it was associated with central catherisation. The sites of thrombosis were portal vein (15), right renal vein (one), right femoral vein (one), multiple veins (one), right femoral artery (3), right iliac artery (2), bilateral iliac and renal arteries (one) and left renal artery (one). Hereditary thrombotic mutations were detected in three patients and anticardiolipin antibody was detected in one, none of whom had been catheterised. The remaining three non-catheterised patients had perinatal risk factors. Venous catheter placement was undertaken in 12 patients (48%), eleven of whom had: umbilical venous catheterisation for exchange transfusion (9), partial exchange transfusion (one) and venous access (one), and one had femoral venous catheterisation for an angiographic study. Arterial catheterisation was undertaken in seven patients (28%) (one infant had both umbilical venous and arterial catheters) for angiographic studies (5) and blood sampling (2). Of the 18 catheterised patients (72%), thrombophilic studies were undertaken in 13 and none had abnormal results. Additional perinatal risk factors were present in 18 patients (72%) and included prematurity (8), congenital heart disease (8), septicaemia (5), dehydration (3), respiratory distress syndrome (3), polycythemia (2), meconium aspiration syndrome (one), pneumonia (one), maternal diabetes (one), necrotising enterocolitis (one) and perinatal asphyxia (one). Although most of the patients recovered after anticoagulant or fibrinolytic therapy, the five (20%) deaths were associated mainly with underlying diseases. Conclusion: The most important risk factor for thrombo-embolic events in neonates is placement of central catheters and some perinatal prothrombotic conditions. Nevertheless, hereditary or acquired thrombophilic risk factors may also be a cause of thrombo-embolism

PARVOVIRUS B19 INFECTION REMINISCENT OF MYELODYSPLASTIC SYNDROME IN THREE CHILDREN WITH CHRONIC HEMOLYTIC ANEMIA

The authors have seen transient pancytopenia with erythroid hypoplasia and striking trilineage myelodysplasia reminiscent of true myelodysplastic syndrome (MDS) in 3 children, 1 with thalassemia intermedia and the other 2 with previously undiagnosed hereditary spherocytosis. In these 3 children transient pancytopenia and myelodysplasia coincided with serological evidence of acute parvovirus B19 (PV-B19) infection, strongly suggesting their relevance. It is of interest that these 3 cases were encountered within a period of 6 months. This might be an incidental event, but it might also be concluded that acute PV-B19 infection associated transient pancytopenia with morphological appearance of MDS may occur more frequently than reported in the literature. So, PV-B19-associated nonclonal MDS should be considered in the differential diagnosis of trueclonal MDS.

Osteopetrosis and Glanzmann's thrombasthenia in a child

Autosomal recessive osteopetrosis is a rare, fatal disease characterized by accumulation of excessive bone mass due to defective bone resorption. The pathogenesis of osteopetrosis is controversial. Osteoblast-osteoclast interaction defects, incorrect differentiation of osteoclasts, abnormal contact between osteoclast and extracellular matrix, and abolished signaling are included in this process. Recently, mutations in the gene of the vacuolar proton pump have been described in some cases of recessive osteopetrosis. Glanzmanns thrombasthenia (GT) is a rare hereditary qualitative platelet disorder characterized by a lifelong bleeding tendency due to quantitative and qualitative abnormalities of the platelet integrin αIIbβ3. Several mutations on either integrin αIIb [glycoprotein (GP) IIb] or integrin β3 (GP IIIa) were reported in GT. We report on a patient with autosomal recessive osteopetrosis concurrently diagnosed with variant type Glanzmanns thrombasthenia. To our knowledge, our patient was the first case reported in the literature in which osteopetrosis and Glanzmanns thrombasthenia were diagnosed together.

The Role of Prohepcidin in Anemia Due to Helicobacter pylori Infection

Background: Hepcidin, a key regulator of iron homeostasis, increases when inflammation and some infections occur. It plays a critical role in macrophage iron retention, which underlies inflammation/infection caused anemia. It is known that Helicobacter pylori (HP) may lead to iron deficiency (ID) due to occult blood loss or reduced iron absorption. This study investigates the role of prohepcidin, hepcidins precursor, in ID and ID anemia (IDA) with a concurrent HP infection. Methods: In this prospectively designed study, 15 patients with IDA and a concurrent HP infection (group 1), 11 patients with an ID and a concurrent HP infection (group 2), and 18 patients with HP infection (group 3) were observed. All groups received only HP eradication therapy. Twenty-five age- and sex-matched children without ID/IDA and HP infection were included in the study as the control group. In all groups and control group, measurements were taken for pre- and posttreatment hemoglobin, serum prohepcidin, serum ferritin, serum iron (SI), transferrin saturation, erythrocyte sedimentation rate, fibrinogen, and C-reactive protein levels. Results: The pretreatment prohepcidin levels were significantly higher only in group 1 compared to the control group (P < .05). In group 1, a significant increase in hemoglobin and SI levels and a significant reduction in prohepcidin levels were additionally observed following HP eradication treatment (P < .05). However, in groups 2 and 3, significant differences in hemoglobin, iron, and prohepcidin levels between pre- and posttreatment were not observed. Conclusion: Elevated serum prohepcidin might indicate the role of inflammation in the etiology of anemia concurrent with HP.

ACUTE RENAL FAILURE DURING ATRA TREATMENT

All-trans-retinoic acid (ATRA), which is used in acute promyelocytic leukemia, is usually well tolerated, but some side effects can be observed. Retinoic acid syndrome is the most severe side effect. Triazole derivatives such as fluconazole inhibit the NADPH-dependent cytochrome P-450-mediated catabolism of ATRA and are increased plasma levels of ATRA. Here, the authors report a case of APL who developed acute renal failure during ATRA and concurrent use of fluconazole.

CMV-induced immune thrombocytopenia and excessive hematogones mimicking an acute B-precursor lymphoblastic leukemia

Hematogones (B-lymphocyte progenitor-precursor cells) are normal bone marrow constituents which are morphologically distinct lymphoid cells with homogeneous, condensed uniform nuclear chromatin and scant cytoplasm. Hematogones can be observed in large numbers in the bone marrow of children with a variety of hematologic and nonhematologic disorders.We present here a 3.5-month-old boy with cytomegalovirus (CMV)-induced immune thrombocytopenia and excessive hematogones in the bone marrow mimicking an acute B-precursor lymphoblastic leukemia. To our knowledge this is the first case of acute CMV infection-induced immune thrombocytopenia with hematogones.

ORAL AND DENTAL FINDINGS IN FANCONI'S ANEMIA

Fanconis anemia is an autosomal recessive disorder characterized by progressive pancytopenia and congenital malformation of the skeleton. This study investigated the oral health status of 15 children with Fanconis anemia, including oral lesions, gingival and periodontal status, and dental abnormalities. All children in the group were found to have a tendency to develop tooth decay and were in need of dental treatment. Two had aggressive periodontitis. In one patient supernumerary teeth were found, while in another teeth were congenitally missing. The increased tendency toward periodontal disease in patients with Fanconis anemia may be due not only to the anemia, leukopenia, and defective detoxification of oxygen radicals that are characteristic of the disease itself, but also to medications applied during intense immunosuppressive treatment, such as prednisolone.