Iranian Journal of Pediatric Hematology and Oncology
Volume:14 Issue: 1, Winter 2024

This study aims to assess the epidemiological, clinical, and paraclinical characteristics and survival of childhood with malignant disorders in the pediatrics department, menoufia University Hospital.

A retrospective study with clinical and epidemiological data from patients was conducted on 314 children who attended Pediatric Department, Haematology-Oncology Unit, Menoufia University Hospital during the last fifteen years.

314 children were assessed, their ages ranged from 2 months-18 years with mean 5.96±3.79 years. Also, 252 (80.3%) were diagnosed with hematological malignancies, and 62 (19.5%) were diagnosed with solid tumors. Among hematological malignancies, 186 were diagnosed with acute leukemia, 158 (49.7%) with acute lymphoblastic leukemia (ALL), and 28 (8.8%) with acute myeloid leukemia (AML). The most frequent clinical presentations were fever in 95.24% in hematological malignancies vs 48.4% in solid (p<0.001),, pallor in 92.5% in hematological malignancies vs 69.4% in solid (p<0.001), hepatomegaly in 81.3% in hematological malignancies vs 37.1% in solid (p<0.001),, lymphadenopathy in 77.6 % in hematological malignancies vs 24.2% in solid (p<0.001), and splenomegaly in 76.3% of hematological malignancies vs 12.9% in solid (p<0.001),The majority of the patients 64.15% had white blood cells (WBCs) less than 50,000/mm³, while 35.85% had WBCs more or equal to 50,000/mm³ with significant relation with risk stratification (p=0.001). The survivors who finished their treatment course were 31.8% and the recurence patients were 9%.

Acute lymphoblastic leukemia is the most frequent childhood hematological neoplasm. Various clinical and laboratory features present at the time of initial diagnosis can predict the likelihood that a patient will remain in remission or not including age: under 1 and over 10 years, gender: male sex, WBCS more than 50,000/mm³ at presentation.

Acute lymphoblastic leukemia (ALL) is the most common cancer among children. The prognostic significance of the cluster of differentiation 34 (CD34) markers in children with B-cell acute lymphoblastic leukemia (B-ALL) is not yet fully understood.

This study is a case-control trial based on the clinical data of 40 children with B-ALL who referred to a pediatric oncology center in the city of Sari, Iran. The data were derived from the demographic findings, laboratory test results at diagnosis, immunophenotyping, transfusion of blood products including packed red blood cells and platelet concentrates, and the frequency and duration of hospitalization due to febrile infection.

Of the participants, 42.5% were CD34-negative and 57.5% were CD34-positive. The mean age of the patients at diagnosis was 3.1 ± 3.3 years (Range:0.1-13.3 years). Also, 60.9% of the CD34-positive children and 47.1% of the CD34-negative ones were boys (P = 0.38). According to the calculated Cohen's d, the relationship of CD34 positivity with transfused packed red blood cell and platelet concentrates was mild -0.15 (95% CI -0.78 to 0.47) (P = 0.55) and moderate 0.49 (95% CI -0.15 to 1.12) (P = 0.29), respectively, which was significant in neither case. Moreover, the relationship of CD34 positivity with the hospitalization frequency of -0.51 (95% CI -1.14 to 0.13) (P = 0.22) and the hospitalization duration of -0.52 (95% CI -1.16 to 0.12) (P = 0.27) due to febrile infection was moderate to strong.

The CD34-positive children with B-ALL experienced less blood products transfusion (except packed red blood cells) and febrile infection in terms of both the frequency and duration of hospitalization during chemotherapy. Therefore, CD34 expression in the B-ALL children was associated with better prognosis.

Idiopathic thrombocytopenic purpura (ITP) is a rare and autoimmune disorder determined by an abnormal reduction in the number of platelets. The current study aims to evaluate the oxidative stress status of children with ITP in two treatment methods using methylprednisolone and methylprednisolone with intravenous immunoglobulin (IVIG).

This retrospective study was conducted on 60 children with ITP who referred to Baghaei Hospital in Ahvaz in 2021.  All the ITP children were equally divided into two groups, 30 receiving methylprednisolone and 30 receiving methylprednisolone and IVIG. The sampling of the patients’ blood was done in two stages before and after the start of treatment. Then, malondialdehyde (MDA), superoxide dismutase (SOD), total antioxidant status (TAS), total oxidative status (TOS), catalase (CAT) and glutathione were measured according to the instructions in the commercial kit. The analyses were performed using SPSS software version 23. P value < 0.05 was significant.

The number of platelets after treatment in methylprednisolone and methylprednisolone+ IVIG groups was 133.44 ± 18.93 and 158.76 ± 34.76 (×103/µL), respectively. Itas significantly increased compared to that before the treatment (P = 0.04). The amount of TAC in the group receiving methylprednisolone + IVIG and the methylprednisolone group was 1.64 ± 0.18 and 1.26 ± 0.53 nm, respectively; there was a remarkable difference between the two groups (P = 0.001). Also, SOD, CAT and glutathione in the methylprednisolone + IVIG group were remarkably higher than those in the methylprednisolone group (P < 0.05). Finally, the levels of TOS were lower in the methylprednisolone + IVIG group (19.74 ± 9.93 μmol) than in the methylprednisolone group (26.65 ± 10.64 μmol) (P = 0.01).

A combination of IVIG and methylprednisolone was found to have a greater effect on improving antioxidant status and decreasing the oxidative stress indices of ITP children.

Immunologic Thrombocytopenic Purpura (ITP) is considered as one of the common diseases among children. The aim of this study is evaluating the treatment indices of ITP in pediatric patients.

In this observational follow-up study, 123 ITP patients were assessed in term of medical history, physical examination, and laboratory tests based on the type of treatment.

Among 123 ITP patients, 70 (56.9%) were female and 53 (43.1%) were male with mean age of 4 years. Considering the platelet count of > 20,000, 115 (93.6%), 4 (3.3%) were treated in less than a month (acute) and 1-6 months (sub-acute). Thirty two patients (26%) did not reach the normal platelet count in 6 months (chronic). IVG, steroid, RhoGAM, steroid+ IVIG, RhoGAM + IVIG, RhoGAM+steroid+IVIG therapy was done in 10.6, 15.4, 2.4, 41.5, 4.1, and 26, respectively. Three patients did not receive any medication. There was no significant relationship between the onset of clinical symptoms and the onset of treatment based on 20,000 platelet count; however, regarding the platelet count of 150000, the relationship was statistically significant. The frequency of ITP was higher in females. There was no report on Intra-cerebral hemorrhage (ICH). In addition, 11 patients (8.9%) were provided with splenectomy. The treatment with combinational therapy of RhoGAM and IVIG was regarded as the highest treatment rate. In addition, the highest length of hospotalization based on initial treatment belonged to steroid treatment followed by the combinational therapy of steroid and IVIG. The patients receiving IVIG were the ones with the highest cost for the first 24 hours of treatment, and regarding the later hospitalization, the patients treated with steroid and combinational therapy of steroid+IVIG had to pay the highest medical expenses.

No significant relationship between the symptoms, platelet count, and the type of treatment.

Various methods have been used to isolate red blood cell (RBC) membrane antigens. In this regard, obtaining the antigen and preserving its structure is of special importance. However, limited studies have been conducted to purify cellular membrane antigens such as Rh proteins.

In this experimental study, Rhc antigens of the RBC membrane was purified. Here, the RBC membrane was solubilized through the lysis buffer. Next, dialysis and affinity chromatography were performed using polyclonal anti-human RhCcEe antibody to isolate Rhc/e antigens from the RBCs with the following blood group characteristics: Rhc+, RhC-, Rhe+, and RhE-. The purified proteins were evaluated by sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE) and dot blot methods. The immunization process was performed in Balb/c mice using the Rhc antigen as an immunogen. After the last injection, the mouse serum was used to titrate antibodies.

Protein bands of the purified antigen were observed in the silver-stained SDS-PAGE gel (region of 25-35 kDa). The OD405nm = 0.56 ± 0.05 results showed the reactivity with Rhc antibody. The specificity of the purified protein was evaluated using the dot blot assay. The anti-sera titration was greater than 1/10,000 against Rhc-coated microwells. Rh antigens can be isolated from the RBC membrane using the non-ionic NP-40 detergent and affinity chromatography.

The Rh antigen can be isolated from the RBC membrane with proper purity by solubilization with the non-ionic NP-40 detergent and purification by affinity chromatography. It seems that the membrane antigen maintains its antigenicity and structure. As a result, it can be detected by blood group-specific antibodies used in the hemagglutination method. Purified antigens may be used to generate antibodies or to study the protein structure.

  For patients with acute myeloid leukemia (AML), the long-term survival rate is still very low. This study examines the effects on AML cell lines of an indole chemical in its free and liposomal forms.

In this experimental case control study, an AML-originated KG-1 cell line was cultured in RPMI 1640 medium. The cells were treated with the free and liposomal forms of an indole compound (C18H10N2F6O) at different concentrations of 20, 40, 100, 200, and 400 µg/mL after they attained the proper confluence. The cellular metabolic activity was examined by an MTT assay. The expression of BAX and BCL-2 genes was investigated by q-PCR to assess the apoptotic effect of that compound. The analysis was also done between each experimental group and the control group using t-test. P<0.05 was assumed significant.

Based on the MTT assay, the lethal effective dose of free indole was found to be 245.1 µg/ml and 164.8 µg/ml in 24 and 48 hours, respectively. The corresponding values for liposomal indole were 47.2 µg/ml and 40.6 µg/ml. Furthermore, treatment with free and liposomal forms of indole resulted in a decline in the expression level of the BCL-2 gene. However, in the case of the liposomal compound, this decrease was only statistically significant after 48 hours of treatment (P < 0.05). Furthermore, the expression of BAX gene increased after treatment with both free and liposomal forms of indole, but it significantly increased only after treatment with the liposomal compound (p < 0.05).

These results suggest that an indole derivative, especially when liposomal, causes apoptosis in AML cells, hence exhibiting cytotoxic effects. To confirm the potential usefulness of this indole derivative as a therapeutic agent for inhibiting tumor progression in the setting of human malignancies, more studies on physiologically relevant models are necessary.

Cancer is as the second leading cause of death among children in the United States. The mortality rate for cancer has witnessed a decline, dropping from 6.5 per 100,000 in 1970 to 2.3 per 100,000 in 2016. Second malignant neoplasms (SMNs) represent novel primary malignancies emerging after the initial cancer diagnosis, particularly prominent as late effects of cancer therapy in children. The incidence of SMNs sees a substantial increase over time, reaching nearly 10% even a decade after the initial diagnosis. A comparative analysis between the general population and child cancer survivors reveals a six-fold higher risk of developing SMNs among the latter. Various factors contribute to the elevated risk of second cancers, with age, lifestyle, environmental influences, primary cancer treatment, and genetic predisposition playing pivotal roles. Noteworthy risk factors for SMNs in children encompass radiation therapy, chemotherapeutic agents, topoisomerase inhibitors, genetic factors, hematopoietic stem cell transplantation, and ionizing radiation, as elucidated in the present study. Despite these findings, further research is imperative to accurately quantify the risks associated with etiological factors, enabling the identification of individuals at a heightened risk for second cancers and facilitating proactive screening and preventive measures.

Congenital dyserythropoietic anemia (CDA) is a rare disease globally. It is characterized by marked dyserythropoiesis as the name suggests. There are only a few hundred cases of Type- I CDA described sporadically. Here we are presenting two cases of CDA –I which were diagnosed based on a simple examination of bone marrow after ruling out common mimickers. One of our patients presented in the neonatal period while the other child presented at the age of six months. Bone marrow examination of both patients showed dyserythropoiesis, and binucleated erythroblasts with much of karyorrhexis. CDA- type II, paroxysmal nocturnal hemoglobinuria was ruled out among other diseases. Genetic tests could not be done. After diagnosis, both patients were put on lifelong blood transfusion therapy and subsequently on iron chelation for treating iron overload secondary to repeated transfusion. Genetic counseling was done in both cases and a bone marrow transplantation option was offered to both families but could not be done due to the non-availability of matched donors and financial constraints. Both patients are still on regular follow-up from our center and growing well. Our case report highlights the fact that this rare entity of congenital dyserythropoietic anemia can be reliably diagnosed even in a resource-poor setting using a simple investigation of bone marrow examination.