Xeroderma pigmentosum and acute myeloid leukemia: a case report

Background Xeroderma pigmentosum is a rare inherited disease characterized by extreme hypersensitivity to ultraviolet rays and predisposing to cutaneous malignancies that can appear in childhood. These manifestations are often associated with ocular lesions and sometimes with neurological disorders. The association of xeroderma pigmentosum with internal neoplasms such as acute myeloblastic leukemia is not reported with great frequency, which confirms the rarity of this occurrence. Case report A 26-year-old Moroccan women, xeroderma pigmentosum patient, was diagnosed with acute myeloblastic leukemia with a complex karyotype. Due to the adverse risk of the xeroderma pigmentosum association with acute myeloblastic leukemia and the profile of acute myeloblastic leukemia with complex karyotype and monosomy 7, which constitute factors of poor prognosis, as well as the absence of studies conceding the tolerance of the chemotherapy by patients suffering from xeroderma pigmentosum, our patient was put under low-dose cytarabine protocol with granulocyte colony-stimulating factor. Unfortunately, she died on the tenth day of chemotherapy by acute pulmonary edema of cardiogenic pace complicated by tamponade. Conclusion According to reports, it is the second case showing association of xeroderma pigmentosum with acute myeloblastic leukemia. The management of these patients remains a challenge. Studies focusing on xeroderma pigmentosum patients developing hematological malignancies are necessary to better understand the most appropriate strategies and precautions for this specific case.

Immune Reconstitution Inflammatory Syndrome Associated with Hepatosplenic Candidiasis in a Patient with Acute Myeloblastic Leukemia: Possible Pathogenesis and Treatment in the Light of Current Knowledge

Acquired immune deficiencies caused by different etiologies, promote invasive fungal infections. When this immunity begins to improve, it can induce an excessive inflammatory response defined as Immune Reconstitution Inflammatory Syndrome (IRIS). Hepatosplenic Candidiasis (HSC) can be considered a form of IRIS syndrome as it occurs following neutrophil recovery in patients treated for acute leukemia. Differentiating IRIS from a single fungal infection or treatment failure due to a similar clinical picture is a real diagnostic problem. Misdiagnosis and subsequently ineffective treatment with antifungal therapy instead of anti-inflammatory drugs, may lead fatal course of the disease. A deep and prolonged neutropenia developed after the first induction chemotherapy in our two and a half-year-old male patient who was followed up in our clinic with the diagnosis of Acute Myeloblastic Leukemia (AML). Our patient had fever, abdominal pain as well as his Gamma Glutamyl Transferase (GGT) and Alkaline Phosphatase (ALP) levels increased during neutropenia recovery. He was diagnosed with hepatosplenic candidiasis, by observing ‘target like abscesses’ on dynamic Magnetic Resonance Imaging (MRI) taken for his newly developing symptoms and laboratory findings while recovering neutropenia. After his first and third induction chemotherapy courses, his fever persisted although antifungal therapy, steroid treatment was initiated considering IRIS. After his re-intensification course, because of the same flare-up symptoms, we started immunglobulin in addition to steroid. With methylprednisolone and intravenous immunoglobulin, his symptoms improved and significant regression was observed in the lesions ‘target-like abscesses’ on MRI and in the laboratory values. Result: IRIS should be considered for patients with hepatic candidiasis whose have persistent fever despite appropriate antifungal therapy. Glucocorticoid should be started first for an anti-inflammatory effect.

#23: Survival Analysis of Childhood Acute Myeloblastic Leukemia in a Limited Resource Center: Do Infection and Lymphocyte-Mediated Immune Response Play a Role?

Background Acute myeloblastic leukemia (AML) is the second most prevalent cancer among Indonesian children. In contrast to those of acute lymphoblastic leukemia (ALL), a number of studies reported unfavorable outcomes following the initiation of cytostatic protocols for childhood AML. In addition, our previous study showed that Gram-negative bacterial infection constitutes the major cause of treatment-related mortality in children with AML. These findings raised a question on the prognostic role of diagnostic lymphopenia, which represents the immunosuppressive state, as demonstrated in adult patients with solid tumors. Methods A retrospective cohort study, which involved children younger than 18 years of age with the newly diagnosed, non-M3 AML, was conducted. These children were admitted to Dr. Sardjito General Hospital, Yogyakarta, Indonesia, during the period of 2011–2019 and received National Pilot Protocol (2011–2016), Pediatric AML Protocol (2016–2018), and International Society of Pediatric Oncology (SIOP) Pediatric Oncology in Developing Countries (PODC) Protocol with prephase (2018–2019). One-year overall survival (OS) was analyzed, using diagnostic absolute lymphocyte counts (ALC0) of more than 4.5 × 109/L and less than 4.5 × 109/L as the determinants, in accordance with those reported in a number of studies on adult AML. Results Sixty-five children, which consisted of 41 (63%) children with ALC0 of more than 4.5 × 109/L and 24 (37%) children with ALC0 of less than 4.5 × 109/L, were eligible for this study. In a multivariate logistic regression analysis, compared with those of diagnostic white blood cell and peripheral blood myeloblast counts, treatment-related pneumonia, gastrointestinal tract infection, and sepsis were the most responsible factors for the occurrence of death within the first year of treatment [relative risk (RR), 2.82; 95% confidence interval (95% CI), 0.99–8.04; P = 0.05]. OS analysis showed that 43% and 16% of children with ALC0 of more than 4.5 × 109/L and less than 4.5 × 109/L, respectively, were alive within the first year of treatment. Cox-regression analysis, however, failed to reach statistical significance [hazard ratio (HR), 0.63; 95% CI, 0.33–1.18; P = 0.15]. Conclusion Despite lacked statistical significance, our study showed the superior one-year OS in children with ALC0 of more than 4.5 × 109/L. Our findings, therefore, might indicate the prognostic role of infection and lymphocyte-mediated immune response in childhood AML.

#8: Survival Analysis in Childhood Acute Myeloblastic Leukemia: Do Infection and Lymphocyte-Mediated Immune Response Play a Role?

Background Acute myeloblastic leukemia (AML) is the second most prevalent cancer among Indonesian children. In contrast, to those of acute lymphoblastic leukemia (ALL), a number of studies reported unfavorable outcomes following the initiation of cytostatic protocols for childhood AML. In addition, our previous study showed that Gram-negative bacterial infection constitutes the major cause of treatment-related mortality in children with AML. These findings raised a question on the prognostic role of diagnostic lymphopenia, which represents the immunosuppressive state, as demonstrated in adult patients with solid tumors. Methods A retrospective cohort study, which involved children younger than 18 years of age with the newly diagnosed, non-M3 AML, was conducted. These children were admitted to Dr. Sardjito General Hospital, Yogyakarta, Indonesia, during the period of 2011–2019 and received National Pilot Protocol (2011–2016), Pediatric AML Protocol (2016–2018), and International Society of Pediatric Oncology (SIOP) Pediatric Oncology in Developing Countries (PODC) Protocol with prephase (2018–2019). One-year overall survival (OS) was analyzed, using diagnostic absolute lymphocyte counts (ALC0) of more than 4.5 × 109/L and less than 4.5 × 109/L as the determinants, in accordance with those reported in a number of studies on adult AML. Results Sixty-five children, which consisted of 41 (63%) children with ALC0 of more than 4.5 × 109/L and 24 (37%) children with ALC0 of less than 4.5 × 109/L, were eligible for this study. Of these 65 children, 56 (86%) children received Cytosine arabinoside in the first week of treatment (National Pilot Protocol and Pediatric AML Protocol), while 9 (14%) children did not (SIOP PODC Protocol with prephase). OS analysis showed that 44% and 19% of children with ALC0 of more than 4.5 × 109/L and ALC0 of less than 4.5×109/L, respectively, were alive within the first year of treatment. Cox-regression analysis, however, failed to reach statistical significance [hazard ratio (HR), 0.63; 95% confidence interval (95% CI), 0.33–1.18; P = 0.15]. Conclusion Despite lacked statistical significance, our study showed the superior one-year OS in children with ALC0 of more than 4.5 × 109/L. Our findings, therefore, might indicate the prognostic role of infection and lymphocyte-mediated immune response in childhood AML.

Cytomegalovirus Keratitis in Acute Myeloblastic Leukemia Report of a Case

Objectives: Here, we report a rare case of acute myeloblastic leukemia (AML) developed a bilateral dendritic epithelial keratitis without retinitisCase presentation: A 58-year-old woman presented to the emergency department of Baqiyatallah Hospital in Tehran due to dyspnea and dry cough along with weakness, lethargy and weight loss resulting from acute myeloblastic leukemia. She was treated for systemic problem. In a while after developing pain, burning, redness of the eyes and ocular mucopurulent discharge, ocular symptoms began. The patient was initially treated with oral acyclovir with a possible diagnosis of Herpes simplex virus Keratitis. Polymerase Chain Reaction (PCR) was performed on ocular discharge specimens collected by soft-tipped applicators reported as CMV. Then, acyclovir was discontinued and bilateral CMV keratitis treated with IV Ganciclovir and her epithelial lesions gradually disappeared. Conclusions: CMV is capable of generating corneal epithelial engagement without corneal endothelium and retina involvement and demonstrated that CMV keratitis can be a rare cause but an emergent problem of acute myeloblastic leukemia (AML). Therefore, in any cases with bilateral corneal herpes keratitis, the patient should be evaluated for immune system deficiency.