Acute renal failure: A rare initial presentation of acute lymphoblastic leukemia

Acute lymphoblastic leukemia has several presentations associated with bone marrow and extramedullary involvement. The unusual presentation may be due to the infiltration of leukemic cells in any organ. An 11-year-old girl presented with fever and vomiting, since one day before admission after starfish biting during swimming. Her vital signs were: blood pressure 150/100 mmHg, pulse 98 beats per minute, respiration 18 breathes per minute, and temperature 37.2 °C (99 F). Laboratory work-up showed blood urea nitrogen 38 mg/dl and creatinine 2.8 mg/dl. In peripheral blood smear, few atypical cells, mild anemia (Hb: 9.2 g/dl), and mild thrombocytopenia (Platelet: 109,000/µL) were detected. Bone marrow aspiration and immunophenotyping were in favor of acute precursor B cell type lymphoblastic leukemia. The patient had a favorable response to treatment after initiating high-risk chemotherapy. Therefore, acute renal failure can be a rare initial presentation of acute lymphoblastic leukemia, and azotemia will improve with an early chemotherapy treatment

A novel frame-shift deletion in FANCF gene causing autosomal recessive Fanconi anemia: A case report

Background: Fanconi anemia (FA) is a heterogeneous genetic disorder characterized by congenital anomalies, early-onset bone marrow failure, and a high predisposition to cancers. Up to know, different genes involved in the DNA repair pathway, mainly FANCA genes, have been identified to be affected in patients with FA. Case presentation: Here, we report clinical, laboratory and genetic findings in a 3.5-year-old Iranian female patient, a product of a consanguineous marriage, who was suspicious of FA, observed with short stature, microcephaly, skin hyperpigmentation, anemia, thrombocytopenia and hypo cellular bone marrow. Therefore, Next Generation Sequencing was performed to identify the genetic cause of the disease in this patient. Results revealed a novel, private, homozygous frameshift mutation in the FANCF gene (NM-022725: c. 534delG, p. G178 fs) which was confirmed by Sanger sequencing in the proband. Conclusion: Such studies may help uncover the exact pathomechanisms of this disorder and establish the genotype-phenotype correlations by identification of more mutations in this gene. It is the first report of a mutation in the FANCF gene in Iranian patients with Fanconi anemia. This new mutation correlates with a hematological problem (pancytopenia), short stature, and microcephaly and skin hyperpigmentation. Until now, no evidence of malignancy was detected

Parameters of tissue iron overload and cardiac function in patients with thalassemia major and intermedia

Noninvasive T2∗ magnetic resonance imaging (MRI) assessment can stratify the risk of subsequent cardiac dysfunction in β-thalassemia major (TM) and β-thalassemia intermedia (TI) patients. The normal level of N-terminal pro B-type natriuretic peptides (NT-proBNP) can rule out acute heart failure. We aim to investigate the relation of NT-proBNP level, T2∗ MRI, and echocardiographic findings in TM and TI patients. In this cross-sectional study, 41 TM patients, 41 TI patients, and 41 healthy individuals (HI) were enrolled. NT-proBNP level, T2∗ MRI, and two-dimensional echocardiography were assessed for all patients and controls. There was statistically significant correlation between NT-proBNP levels and mitral inflow late diastolic velocity (r = -0.538; p = 0.006) in TM group. There was statistically significant correlation between NT-proBNP levels and tricuspid annulus systolic velocity (r = -0.438; p = 0.028), systolic velocity of septum (r = -0.472; p = 0.020), and mitral inflow early-to-late diastolic wave ratio (r = 0.592; p = 0.002) in TM group. Early diagnosis and treatment of myocardial iron overload are likely to prevent the mortality in patients with established ventricular dysfunction. Since NT-proBNP levels were not significantly increased in documented left ventricular (LV) diastolic dysfunction, this factor may not be sensitive for the detection of latent LV diastolic dysfunction in the early stages of disease progression

Cardioprotective effects of deferoxamine in acute and subacute cardiotoxicities of doxorubicin: a randomized clinical trial

Background: Cardiotoxicity is a major concern following doxorubicin (DOX) use in the treatment of malignancies. We aimed to investigate whether deferoxamine (DFO) can prevent acute cardiotoxicity in children with cancer who were treated with DOX as part of their chemotherapy. Results: Sixty-two newly-diagnosed pediatric cancer patients aged 2–18 years with DOX as part of their treatment regimens were assigned to three groups: group 1 (no intervention, n = 21), group II (Deferoxamine (DFO) 10 times DOX dose, n = 20), and group III (DFO 50 mg/kg, n = 21). Patients in the intervention groups were pretreated with DFO 8-h intravenous infusion in each chemotherapy course during and after completion of DOX infusion. Conventional and tissue Doppler echocardiography, serum concentrations of human brain natriuretic peptide (BNP), and cardiac troponin I (cTnI) were checked after the last course of chemotherapy. Sixty patients were analyzed. The level of cTnI was < 0.01 in all patients. Serum BNP was significantly lower in group 3 compared to control subjects (P = 0.036). No significant differences were observed in the parameters of Doppler echocardiography. Significant lower values of tissue Doppler late diastolic velocity at the lateral annulus of the tricuspid valve were noticed in group 3 in comparison with controls. By using Pearson analysis, tissue Doppler systolic velocity of the septum showed a marginally significant negative correlation with DOX dose (P = 0.05, r = − 0.308). No adverse effect was reported in the intervention groups. Conclusions: High-dose DFO (50 mg/kg) may serve as a promising cardioprotective agent at least at the molecular level in cancer patients treated with DOX. Further multicenter trials with longer follow-ups are needed to investigate its protective role in delayed DOX-induced cardiac damage. Trial registration IRCT, IRCT2016080615666N5. Registered 6 September 2016

Gynecomastia as a late complication of childhood cancer and its treatment that can affect the quality of life of male survivors

Childhood cancer is relatively rare, and nowadays it is curable in more than 80% of children. Childhood cancer therapy is directed not only at improving survival but recently, we also concentrate on reducing late effects. We want children who have a diagnosis of cancer to survive and have an excellent quality of life. Gynecomastia and fertility outcome of the survivors of childhood malignancies should be considered in the follow-up of teen agers and young adults and should be approached in an accurate manner and managed in comprehensive teams

Successful treatment of refractory metastatic neuroblastoma with panobinostat in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine therapy

Introduction: Neuroblastoma commonly required multimodal therapy containing surgery, chemotherapy, radiotherapy, and immunotherapy. Case report: In our case, who had refractory metastatic neuroblastoma, we use histone deacetylase inhibitor (panobinostat) in combination with chemotherapy agents and iodine-131-meta-iodobenzylguanidine (MIBG) therapy. Management and outcome: This approach leads to successfully treat the patient. MIBG scan and bone marrow examination after therapy revealed no evidence of tumor. Now, she underwent autologous transplantation six months ago and free of tumor. Conclusion: Panobinostat can cause apoptosis induction in refractory metastatic neuroblastoma in combination with MIBG therapy and chemotherapy

Deferoxamine protective effect in preventing nephrotoxicity in childreunder treatment with doxorubicin: A randomized clinical trial

Background: Nephrotoxicity secondary to doxorubicin (DOX) may be associated with high morbidity and mortality rates. We aimed to assess the efficacy of Deferoxamine (DFO) in preventing DOX-induced nephrotoxicity in pediatric malignancy. Methods: This Parallel-group randomized clinical trial was done on 62 children aged 2-18 years who had new onset malignancy treated with DOX. They were randomly assigned in three groups; group 1 (no intervention, n=21), group II (DFO 10 times DOX dose, n=20), group III (DFO 50mg/kg, n=21). Patients in the intervention groups received DFO concomitant with DOX 8-hour intravenous infusion in each chemotherapy course. Blood urea nitrogen, serum creatinine, electrolytes, calcium, phosphorus, magnesium and albumin levels, urine microalbumin, urine protein/creatinine ratio, and urine N-acetyl-β-D-glucosaminidase (NAG) as well as findings of kidney ultrasonography were compared between the groups after the last course of chemotherapy. The primary outcome was to compare the radiologic and serologic markers of glomerular and tubular damage between the 3 groups. Results: Sixty patients were analyzed. Patients treated with DFO 10 times the dose of DOX had significantly lower urine NAG level compared to the control group (P=0.032). No significant renal damage was reported in their ultrasonography in the 3 groups. DFO was safely tolerated without any adverse effect. Conclusion: DFO with 10-times the DOX dose may effectively prevent DOX-induced nephrotoxicity at least at the molecular level. Increasing the dose of DFO is not accompanied by better efficacy

Feasibility and Therapeutic Potential of 177Lu-Fibroblast Activation Protein Inhibitor-46 for Patients With Relapsed or Refractory Cancers: A Preliminary Study

INTRODUCTION: Fibroblast activation protein (FAP) is a member of the serine protease family and has a high expression in the stroma of approximately 90% of epithelial malignancies. The present investigation aimed to assess the feasibility, safety, and dosimetry data of 177Lu-FAPI-46 in diverse malignancies. PATIENTS AND METHODS: Patients with advanced cancers with nonoperable tumors, or tumors refractory to conventional therapies, were enrolled. Treatment included escalating doses of 177Lu-FAPI-46 (1.85-4.44 GBq) per cycle using a combination of clinical and statistical expertise design, and intervals of 4 to 6 weeks were considered between the cycles. Biodistribution and dosimetry were examined by whole-body scans. We applied the National Cancer Institute Common Terminology Criteria for Adverse Events version 4.03 to measure peptide-targeted radionuclide therapy (PTRT)-associated toxicity. RESULTS: A total of 21 patients (11 females and 10 males) with a median age of 50 years (range, 6-79 years) were investigated. Of 21 participants, 18 cases were selected for PTRT. Overall, 36 PTRT cycles were performed. The median number of PTRT cycles and the median injected amount of activity in each cycle were 2 and 3.7 GBq, respectively. The dosimetric analysis revealed median absorbed doses of 0.026, 0.136, 0.886, and 0.02 with ranges of 0.023-0.034, 0.001-0.2, 0.076-1.39, and 0.002-0.2 mGy/MBq for the whole body, liver, kidneys, and spleen, respectively. The therapy was well tolerated in almost all patients. CONCLUSIONS: The findings of this preliminary investigation might indicate the potential feasibility and safety of PTRT using 177Lu-FAPI-46 for different aggressive tumors. Moreover, the current study could be beneficial in determining the suitable amount of activity for a phase 2 study