Idiopathic Hyperammonemia That Developed During Initial Treatment With Steroid in a Patient With Newly Diagnosed Leukemia

Kobayashi, Shogo; Ito, Masaki; Sano, Hideki; Mochizuki, Kazuhiro; Akaihata, Mitsuko; Waragai, Tomoko; Ohara, Yoshihiro; Hosoya, Mitsuaki; Kikuta, Atsushi

doi:10.1097/mph.0000000000000255

Cited by 6 publications

(4 citation statements)

References 17 publications

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“…Although there has been a report of those associated with 5-fluorouracil, ammonia is a known metabolite of 5-fluorouracil, with evidence showing a transient and favorable course (14). Only one case has been reported, in which the disease developed during the early steroid treatment phase of acute lymphocytic leukemia (15). No previous reports have suggested an association between hyperammonemia and mercaptopurine, which is the most recently used chemotherapy drug in the present case.…”

Section: Discussionmentioning

confidence: 63%

Case Report: Juvenile Myelomonocytic Leukemia Underlying Ornithine Transcarbamylase Deficiency Safely Treated Using Hematopoietic Stem Cell Transplantation

et al. 2022

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Background:Juvenile myelomonocytic leukemia (JMML), which is predominantly found in infants, is a clonal abnormality of pluripotent hematopoietic stem cells and presents with the symptoms of both myeloproliferative tumors and myelodysplastic syndromes. Estimates have shown that ~20 cases of JMML occur annually in Japan. Ornithine transcarbamylase deficiency (OTCD), the most common among all urea cycle disorders (UCDs), occurs in 1 of 80,000 people in Japan.Case PresentationA 10-month-old infant who had fever, vomiting, and diarrhea for 2 days was referred to our hospital for the following abnormalities in blood tests: white blood cell count, 48,200/μL; hemoglobin, 9.0 g/dL; and platelet count, 135,000/μL. Bone marrow examination showed a nucleated cell count of 396,000/mm3 and blast cell count of 5.0%, as well as decreased mature granulocyte count and slightly myeloperoxidase stain-negative blasts but no monoclonal cell proliferation on May–Giemsa staining. Colony assay showed the proliferation of spontaneous colony and high sensitivity to granulocyte-macrophage colony-stimulating factor. Genetic analysis of peripheral blood mononuclear cells showed that the patient was positive for neuroblastoma RAS (NRAS) mutation. The patient was ultimately diagnosed with JMML. Approximately 170 days after his first hematopoietic stem cell transplantation (HSCT), the patient's JMML relapsed. Shortly after the recurrence, nausea, vomiting, hyperventilation, and decreased vitality were observed, followed by a decrease in the level of consciousness. The patient's ammonia level was 472 μmol/L. A test for seven different genetic mutations for the UCD showed the presence of c. 119G>A (amino acid change p. Arg40His). As such, late-onset OTCD was added to his diagnosis. Administration of sodium phenylacetate, l-arginine hydrochloride, and carnitine was continued following the diagnosis of OTCD, after which hyperammonemia was not observed. Regarding JMML relapse, HSCT was performed on day 405 after the first transplantation.ConclusionHyperammonemia should be considered a differential diagnosis when unexplained and non-specific symptoms occur during the treatment of hematologic malignancies. Patients should be tested for UCD as a cause of hyperammonemia, and treatment for hyperammonemia should be continued until the cause is identified. The patient shows normal developmental progress, has an intact neurological status, and has not experienced another hyperammonemia attack. His JMML has remained in remission for over 3 years.

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Section: Discussionmentioning

confidence: 63%

Case Report: Juvenile Myelomonocytic Leukemia Underlying Ornithine Transcarbamylase Deficiency Safely Treated Using Hematopoietic Stem Cell Transplantation

et al. 2022

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“…e role of some additional Case Reports in Hematology precipitating factors may also be discussed. Among them, sepsis, dehydration, and nitrogen load by parenteral nutrition are commonly found and were present in our patient [1,9,12]. e ammonia production could also have been increased by the ileus-related microbial proliferation [13].…”

Section: Discussionmentioning

confidence: 64%

Fatal Idiopathic Hyperammonemia after Induction Chemotherapy for Acute Myeloid Leukemia

Angelo

Vincent

Komuta

et al. 2020

Case Reports in Hematology

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Idiopathic hyperammonemia is a rare but potentially fatal complication occurring in patients with acute leukemia or bone marrow transplantation. The role of some specific anticancer drugs may be discussed, but the etiology of hyperammonemia is often multifactorial. We report the case of a 40-year-old woman who developed fatal idiopathic hyperammonemia two weeks after induction chemotherapy with idarubicin-aracytine for acute myeloid leukemia. Despite intensive care management and extrarenal epuration, the patient was declared brain dead two days after hyperammonemia onset.

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“…The occurrence of IHA during initiation of chemotherapy for leukemia is highly unusual and was described in a pediatric population only twice: once on day 15 in a 15year-old male with newly diagnosed acute lymphoblastic leukemia and initiation of chemotherapy with steroids only 14 and another time in a case of a 13-month-old male with a lethal IHA on day 35 after induction therapy with seven different chemotherapeutic drugs. 6 The only report of IHA in combination with mitoxantrone described the case of a 59-year-old woman who received a polychemotherapy including mitoxantrone.…”

Section: Discussionmentioning

confidence: 99%

Transient Hyperammonemic Encephalopathy in a Child with Relapsed Acute Lymphoblastic Leukemia and Severe Tumor Lysis Syndrome

Dubinski

Feuchtinger

Maier

et al. 2021

Journal of Child Science

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Idiopathic hyperammonemia (IHA) is a severe condition, which has been reported in intensive chemotherapy and bone marrow transplantation. This case elucidates the diagnostic dilemma in children undergoing initiation of chemotherapy and developing severe disorders of qualitative and quantitative consciousness in the presence of hyperammonemia (HA) and lactic acidosis. The role of mitoxantrone as a causative agent for IHA in children is elusive. Children undergoing chemotherapy are often in a poor general condition, and the clinical presentation of HA is heterogeneous. This case should be a reminder for clinicians to check for HA in children with tumor lysis syndrome and acute neurological deterioration.

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Idiopathic Hyperammonemia That Developed During Initial Treatment With Steroid in a Patient With Newly Diagnosed Leukemia

Cited by 6 publications

References 17 publications

Case Report: Juvenile Myelomonocytic Leukemia Underlying Ornithine Transcarbamylase Deficiency Safely Treated Using Hematopoietic Stem Cell Transplantation

Case Report: Juvenile Myelomonocytic Leukemia Underlying Ornithine Transcarbamylase Deficiency Safely Treated Using Hematopoietic Stem Cell Transplantation

Fatal Idiopathic Hyperammonemia after Induction Chemotherapy for Acute Myeloid Leukemia

Transient Hyperammonemic Encephalopathy in a Child with Relapsed Acute Lymphoblastic Leukemia and Severe Tumor Lysis Syndrome

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