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.

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.

Spurious Thrombocytosis Caused by Tumor Cell Lysis in a Patient with Acute Monocytic Leukemia.

BACKGROUND: Tumor lysis syndrome can occur after treatment of fast-growing cancers. Early detection of tumor lysis is crucial to minimize the toxic effects on organs and potentially life-threatening complications. METHODS: A patient with acute monocytic leukemia presented with spurious thrombocytosis. A peripheral blood smear was stained with alpha-naphthyl butyrate esterase to discriminate tumor cell fragments from platelets. RESULTS: Peripheral blood smears showed widespread leukemic cell fragmentation. Tumor lysis syndrome (TLS) after treatment for acute monocytic leukemia was diagnosed. The patient underwent chemo- and radiotherapy followed by umbilical cord blood transplantation and remains symptom-free two years after transplantation. CONCLUSIONS: For patients with thrombocytosis accompanied by bizarre scatter-grams on automatic hematologic analyzers, further diagnostic procedures should be performed to determine the exact cause of thrombocytosis.

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.

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.

Dexamethasone in patients with acute lung injury from acute monocytic leukaemia.

The use of steroids is not required in myeloid malignancies and remains controversial in patients with acute lung injury (ALI) or acute respiratory distress syndrome (ARDS). We sought to evaluate dexamethasone in patients with ALI/ARDS caused by acute monocytic leukaemia (AML FAB-M5) via either leukostasis or leukaemic infiltration. Dexamethasone (10 mg every 6 h until neutropenia) was added to chemotherapy and intensive care unit (ICU) management in 20 consecutive patients between 2005 and 2008, whose data were compared with those from 20 historical controls (1994-2002). ICU mortality was the primary criterion. We also compared respiratory deterioration rates, need for ventilation and nosocomial infections. 17 (85%) patients had hyperleukocytosis, 19 (95%) had leukaemic masses, and all 20 had severe pancytopenia. All patients presented with respiratory symptoms and pulmonary infiltrates prior to AML FAB-M5 diagnosis. Compared with historical controls, dexamethasone-treated patients had a significantly lower ICU mortality rate (20% versus 50%; p = 0.04) and a trend for less respiratory deterioration (50% versus 80%; p = 0.07). There were no significant increases in the rates of infections with dexamethasone. In conclusion, in patients with ALI/ARDS related to AML FAB-M5, adding dexamethasone to conventional chemotherapy seemed effective and safe. These results warrant a controlled trial of dexamethasone versus placebo in AML FAB-M5 patients with noninfectious pulmonary infiltrates.

Leukemia cutis amidst a psoriatic flare: a case report.

Leukemia Cutis (LC) has many clinical morphologies that present a diagnostic challenge. This case report of a 58-year-old man experiencing a flare of psoriasis elucidates the need for clinical suspicion when a history of leukemia is present. A skin biopsy revealed histopathologic findings of psoriasis and an infiltrate of mononuclear cells consistent with LC. Upon review of the literature, 2 additional cases were reported of concurrent psoriasis and LC.

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.

Granulocytic sarcoma of the knee and the spine as aleukemic relapse of acute myeloid leukemia in a child.

Granulocytic sarcoma is a very rare disease in childhood, which may precede the clinical manifestations of acute myeloid leukemia or may occur as a relapse of a nonleukemic acute myeloid leukemia. It is a malignant, solid tumor consisting of myeloblasts or immature myeloid cells occurring in extramedullary sites. We report interesting magnetic resonance images of a nonleukemic relapse of acute myeloid leukemia in a child presenting as a granulocytic sarcoma unusually localized to the knee and spine.

Life-threatening complications associated with acute monocytic leukaemia after dental treatment.

It is highly recommended to conduct a prophylactic check for any dental problems on patients who suffer from leukaemia before chemotherapy begins. Bacteraemia caused by oral microflora may be very dangerous for patients with haematological malignancies. However, it should be noted that the prophylactic process itself might bring about life-threatening complications if there is only a short interval between dental treatment and the beginning of chemotherapy, or if the dental treatment is too aggressive. We present a case where this prophylactic procedure produced life-threatening complications for a patient with acute myeloid leukaemia.

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.

Cutaneous presentation of aleukemic monoblastic leukemia cutis - a case report and review of literature with focus on immunohistochemistry.

Aleukemic monoblastic leukemia cutis is a rare cutaneous manifestation of a systemic hematological disorder associated with dermal infiltration of monoblasts preceding bone marrow or peripheral blood involvement. We report a case of a 75-year-old woman who presented with an erythematous maculopapular rash, which was clinically diagnosed as viral exanthema. Microscopy of the skin biopsy showed features of monoblastic leukemia. Her general physical condition rapidly deteriorated and she died 4 weeks later. We present this case to alert dermatologists of innocuous erythematous skin lesions clinically resembling a viral exanthema, which, in rare instances, may be a presenting feature of an aleukemic monoblastic leukemia cutis. This entity poses problems for dermatopathologists even on immunohistochemistry as monoblasts are negative for hemopoietic precursor cell antigens like CD34, Terminal deoxynncleotidy1 transferase (TdT) and CD117.