Pediatric acute myeloid leukemia and hyperleukocytosis with WBC count greater than 50 × 109/L.

BACKGROUND: Acute myeloid leukemia (AML) and hyperleukocytosis have an unfavorable prognosis, but the impact of hyperleukocytosis on the prognosis of pediatric AML remains uncertain. We investigated the clinical characteristics and prognosis of pediatric AML with hyperleukocytosis, defined as WBC ≥ 50 × 109/L. METHODS: A total of 132 patients with newly diagnosed childhood AML with hyperleukocytosis were consecutively enrolled at our center from September 2009 to August 2021 to investigate prognostic factors and clinical outcomes. RESULTS: Hyperleukocytosis occurred in 27.4% of AML patients. Pediatric patients with hyperleukocytosis had similar CR and OS rates to those without hyperleukocytosis, but had a lower EFS rate. In our study, rates of CR1, mortality, relapsed/refractory disease, and HSCT were comparable between AML patients with WBC counts of 50-100 × 109/L and ≥ 100 × 109/L. AML patients with a WBC count of 50-100 × 109/L had a similar 5-year OS rate to patients with a WBC count ≥ 100 × 109/L (74.6% vs. 75.4%, P = 0.921). Among all patients with hyperleukocytosis, the FAB M5 subtype was associated with significantly inferior survival, and the prognosis of CBF-AML was good. CONCLUSIONS: Pediatric AML patients with hyperleukocytosis have the similar prognosis regardless of whether their WBC count is 50-100 × 109/L or ≥ 100 × 109/L.

Leukemia cutis - A case of cutaneous manifestation of acute monoblastic leukemia.

Leukemia cutis is a comprehensive terminology for dermal manifestations of any type of leukemia either with accompanied or antecedent blood or bone marrow involvement. Although both myeloid and lymphoid neoplastic leukocytes can infiltrate the skin, the frequency is higher among children with congenital myeloid leukemia. However, the underlying pathogenesis of dermal tropism is not yet established. Clinical manifestation varies regarding appearance, site, and numbers. Skin biopsy is essential for the early establishment of the diagnosis and to guide for further testing and categorical management. We report the case of acute myeloid leukemia-cutis in a 22-year-old female where cutaneous manifestation preceded the hematological diagnosis of systemic leukemia.

Rare Case of Refractory Hypokalemia in a Patient with Acute Monocytic Leukemia.

BACKGROUND Refractory hypokalemia has been rarely demonstrated in patients with acute monocytic leukemia (AMoL). Hypokalemia develops in these patients owing to renal tubular dysfunction, secondary to lysozyme enzymes that are released by monocytes in AMoL. Additionally, renin-like substances are produced from monocytes and can lead to hypokalemia and metabolic alkalosis. There is also an entity called spurious hypokalemia, in which high numbers of metabolically active cells in blood samples increase sodium-potassium ATPase activity, resulting in influx of potassium. Additional research is warranted regarding this specific demographic to create standardized treatment approaches to electrolyte repletion. CASE REPORT In this case report, we demonstrate a rare case of an 82-year-old woman with AMoL, complicated by refractory hypokalemia, who presented with concerns of fatigue. The patient's initial laboratory results were significant for leukocytosis with monocytosis and severe hypokalemia. Refractory hypokalemia was noted, despite administration of aggressive repletions. During her hospitalization, AMoL was diagnosed and an extensive workup was performed to evaluate the underlying cause of hypokalemia. Ultimately, the patient died on day 4 of hospitalization. We describe the correlation between severe refractory hypokalemia and leukocytosis and provide a literature review of multiple etiologies of refractory hypokalemia in patients with AMoL. CONCLUSIONS We evaluated the numerous pathophysiologic mechanisms responsible for refractory hypokalemia in patients with AMoL. Our therapeutic outcomes were limited owing to the patient's early death. It is of high importance to evaluate the underlying cause of hypokalemia in these patients and to treat accordingly with caution.

Leukemic retinopathy presenting as concurrent bilateral subhyaloid hemorrhage and subarachnoid hemorrhage in a patient with acute monocytic leukemia: a case report.

BACKGROUND: Ophthalmic manifestations are common in patients with leukemia, developing in nearly 50% of cases. Intracranial hemorrhage is another potentially fatal complication of leukemia. In this case report, we aim to present a challenging case that involves both ophthalmic and intracranial manifestations in an individual with acute monocytic leukemia. CASE PRESENTATION: A 36-year-old Persian male presented to the emergency room with complaints of fever, headache, and bilateral blurred vision. The patient had been diagnosed with acute monocytic leukemia 3 months prior and had undergone four sessions of induction chemotherapy, the last of which was 10 days prior to admission. The patient was admitted to the internal medicine service, and initial lab studies confirmed pancytopenia, including severe neutropenia, anemia, and thrombocytopenia. Subarachnoid hemorrhage in the left frontal lobe was detected through spiral brain computed tomography scan. Ophthalmic examination revealed visual acuity of light perception in the right eye and 3-m finger count in the left eye. Fundus examination revealed bilateral peripapillary subhyaloid and intraretinal hemorrhages, confirming leukemic retinopathy. The patient showed significant improvement in visual acuity and hemorrhage resolution through conservative treatment and regular follow-ups after 3 months. CONCLUSION: Simultaneous subarachnoid hemorrhage and bilateral subhyaloid hemorrhages seemed to have occurred as a result of pancytopenia. Management approach of ophthalmic manifestations of leukemia involves interdisciplinary cooperation and should be individualized on the basis of the patients' underlying medical condition.

Leukemia Cutis With Histopathologic and Immunophenotypic Features Resembling S100-Negative CD1a-Positive Cutaneous Histiocytosis.

ABSTRACT: S100-negative CD1a-positive cutaneous histiocytosis is an exceedingly rare histiocytosis that is defined histopathologically by a dense dermal infiltrate of ovoid mononuclear cells with grooved nuclei and ample cytoplasm with variable nuclear atypia and mitoses that are immunohistochemically positive for CD1a and negative for S100 and CD207 (langerin). The histogenesis of S100-negative CD1a-positive histiocytosis is unclear, and its precursor cell has yet to be characterized. Although all cases thus far have been described as benign and occasionally self-resolving, the clinical course and outcome of this disease are not fully understood. This case expands the spectrum of disease associated with S100-negative CD1a-positive histiocytosis given its malignant course.

Development of myelofibrosis and acute monocytic leukemia in a patient with hereditary spherocytosis: A case report.

INTRODUCTION: Common symptoms of hereditary spherocytosis (HS) include intermittent jaundice and splenomegaly. Here, we present an unusual clinical course wherein a patient with HS treated with splenectomy developed secondary myelofibrosis and acute monocytic leukemia (M5). PATIENT CONCERNS: After presenting with paleness, fatigue and jaundice, the patient was diagnosed with HS. After splenectomy, follow-up testing, including bone marrow biopsy, revealed myelofibrosis. Subsequently, the patient exhibited blood cell abnormalities consistent with M5. DIAGNOSIS: M5 comorbid with myelofibrosis and a history of HS. INTERVENTIONS: HS was treated with splenectomy. Myelofibrosis was treated with hydroxyurea. The patient refused chemotherapy for M5 and was discharged. He was maintained on hydroxyurea and received periodic blood product transfusions with regular routine blood test monitoring. OUTCOMES: Because of intracranial hemorrhage, the patient died on May 17, 2018, a little >10 months after being diagnosed with leukemia. CONCLUSION: The present patient developed M5 while undergoing treatment for myelofibrosis and after undergoing splenectomy for HS, raising the question of whether these conditions might be associated. Examination of this question will require the analysis of additional cases.

Improvement of refractory acyclovir-resistant herpes simplex virus type 1 infection by continuous acyclovir administration.

Resistant herpes simplex virus type 1 (HSV-1) infection is sometimes fatal for immunocompromised patients. Here, we report 10-year-old girl receiving hematopoietic stem cell transplantation developed refractory HSV-1 infection, which was persisted to intermittent acyclovir (ACV) or foscarnet (FOS) administrations but was improved by continuous ACV administration. The isolates from the lesion were identified with low susceptibilities to ACV and FOS by plaque reduction assay due to DNA pol gene mutation. Continuous ACV administration overcomes the efficacy of intermittent administration and could be the best option to treat severe HSV-1 infectious patients.

Clonal dynamics in a case of acute monoblastic leukemia that later developed myeloproliferative neoplasm.

In acute myeloid leukemia (AML), patients may harbor pre-leukemic hematopoietic stem cells (HSCs) containing some, but not all, of the mutations observed in the leukemic cells. These pre-leukemic HSCs may survive induction chemotherapy and contribute to AML relapse by obtaining additional mutations. We report here an acute monoblastic leukemia (AMoL) patient who later developed an unclassifiable myeloproliferative neoplasm (MPN-U). Whole-exome sequencing and cluster analysis demonstrated the presence of three distinct major clones during the clinical course: (1) an AMoL clone with ASXL1, CBL, and NPM1 somatic mutations, likely associated with the pathogenesis, and GATA2, SRSF2, and TET2 mutations, (2) an AMoL remission clone, with mutated GATA2, SRSF2, and TET2 only (possibly the founding clone (pre-leukemic HSC) that survived chemotherapy), (3) a small subclone which had JAK2 mutation during the AMoL remission, appearing at MPN-U manifestation with additional mutations. These findings suggest that pre-leukemic HSCs in AML patients may give rise to non-AML myeloid malignancies. This is the first report to analyze the clonal evolution from AMoL to MPN-U, which may provide new insight into the development of myeloid malignancies.

Whether innate immune together with genetic factor are involved in leukemic arthritis?: A case report and review of the literature.

We report a case of leukemic arthritis (LA) of monocytic differentiation, which presented with spondyloarthritis-like symptoms and a positive human leukocyte antigen-B27, and discuss its potential mechanisms.The patient was admitted because of pain in her right knee and lower back for 18 months. Magnetic resonance imaging showed diffuse hyperintense signal in the bilateral liac bones and bone marrow edema and synovitis in the right knee.The diagnosis of acute monocytic leukemia and LA were concluded by bone marrow aspiration and flow cytometry of the synovial fluid.The patient had poor response to nonsteroidal anti-inflammatory drugs. One week after she received chemotherapy, the symptoms were dramatically relieved.For 5-year follow-up, she got clinical remission without suffering pain of the right knee and the lower back.Leukemic arthritis is a rare manifestation of leukemia with unknown mechanism and may be the initial presentation of leukemia. The problem whether abnormal immune response of the neoplasitc monocytes together with hereditary factors contribute to the pathogenesis of LA in adult is raised from this case, which worth further research.

Atypical Acute Monocytic Leukemia: a Lymphoma Presentation and Association with Complex Karyotype.

A 71-year-old woman presented with acute monocytic leukemia with lymphoma-like morphologic features and an unusual complex karyotype. Bone marrow (BM) aspiration revealed acute monocytic leukemia with lymphoma-like morphologic features. Thus, we called the condition a lymphoma presentation. The patient was diagnosed with acute monocytic leukemia (AML FAB M5a) according to the French-American-British (FAB) classification and the new World Health Organization (WHO) classification [1]. To our knowledge, this is the first reported case of an unusual complex karyotype in acute monocytic leukemia with a lymphoma presentation and adds to the expanding list of karyotypic abnormalities seen in acute monocytic leukemia. We present the case given its rarity, occasional misdiagnosis and poor prognosis. Whether this complex karyotype resulted in such lymphoma-like morphologic features remains to be determined. The case illustrates the importance of the morphologic features cognition and avoiding misdiagnosis. Clinical trials are available to further explore how to extend survival time and contribute to a better prognosis for patients suffering from acute monocytic leukemia with a complex karyotype.

Central hypothyroidism in a pediatric case of primary acute monoblastic leukemia with central nervous system infiltration: A case report.

RATIONALE: Central nervous system (CNS) leukemia is a frequent diagnosis in pediatric acute myeloblastic leukemia (AML) and includes neural symptoms. However, CNS leukemia is rarely associated with central hypsothyroidism. PATIENT CONCERNS AND DIAGNOSES: A 2-year-old female with AML with MLL rearrangement presented with CNS infiltration. Laboratory tests suggested the presence of central hypothyroidism (thyroid-stimulating hormone [TSH]: 0.48 mIU/ml, normal range 0.7-6.4 mIU/ml; serum free thyroxine [FT4]: 0.62 ng/dl, normal range 0.8-2.2 ng/dl; free triiodothyronine: 1.57 pg/ml, normal range 2.7-5.6 pg/ml). Magnetic resonance imaging detected no lesions in the hypothalamus, pituitary, or thyroid. INTERVENTIONS AND OUTCOMES: Levothyroxine (2.5 mg/kg/day) was administered together with chemotherapy and intrathecal injection of methotrexate, cytarabine, and hydrocortisone into the cerebrospinal fluid. The FT4 concentration increased after levothyroxine treatment, but later decreased after relapse of CNS leukemia. The TSH concentrations remained low. After remission of CNS leukemia, the TSH and FT4 concentrations quickly recovered to their normal ranges. LESSONS: We believe that the CNS leukemia directly affected TSH and thyroid hormone secretion in our patient.