Characteristics and treatment of acute myeloid neoplasms with cutaneous involvement in infants up to 6 months of age: A retrospective study.

BACKGROUND: Myeloid neoplasms account for 50% of cases of pediatric leukemias in infants. Approximately 25%-50% of patients with newborn leukemia have cutaneous extramedullary disease (EMD). In less than 10% of patients, aleukemic leukemia cutis or isolated extramedullary disease with cutaneous involvement (cEMD) occurs when skin lesions appear prior to bone marrow involvement and systemic symptoms. Interestingly, in acute myeloid leukemia with cutaneous EMD (AML-cEMD) and cEMD, spontaneous remissions have been reported. METHOD: This is a multicentric retrospective cohort study aiming to describe characteristics, treatment, and outcome of infants with either cEMD or presence of cutaneous disease with involvement of the bone marrow (AML-cEMD). This study included patients born between 1990 and 2018 from Italy, the Netherlands, Switzerland, and the United States, diagnosed between 0 and 6 months of life with cEMD or AML-cEMD. Descriptive statistics, Fisher's exact test, Kaplan-Meier method, and log rank test were applied. RESULTS: The cohort consisted of n = 50 patients, including 42 AML-cEMD and eight cEMD patients. The most common genetic mutation found was a KMT2A rearrangement (n = 26, 52%). Overall 5-year event-free survival (EFS) and overall survival (OS) were 66% [confidence interval (CI): 51-78] and 75% [CI: 60-85], respectively. In two patients, complete spontaneous remission occurred without any therapy. Central nervous system (CNS) involvement was found in 25% of cEMD patients. No difference in outcomes was observed between the AML-cEMD and cEMD groups, but none of the latter patients included in the study died. KMT2A rearrangements were not associated with poorer prognosis. CONCLUSION: In the largest cohort to date, our study describes the characteristics of infants with cutaneous involvement of myeloid neoplasms including cytomolecular findings and survival rates. Further prospective biologic and clinical studies of these infants with myeloid neoplasms will be required to individualize therapy for this rare patient population.

A case of aleukemic leukemia cutis after COVID-19 infection presenting as facial rash.

Aleukemic leukemia cutis (ALC) is a rare condition that is characterized by leukemic cells in the skin before presenting in the peripheral blood or bone marrow. We report a case of a 43-year-old woman who underwent assessment for bilateral facial nodules arising 1 month after COVID-19 infection. A punch biopsy specimen showed a malignant neoplasm primarily composed of immature blasts dissecting through the collagen in the dermis, concerning for myeloid sarcoma versus leukemia cutis. Bone marrow and blood specimens were negative for hematologic malignancy. The patient was appropriately treated with chemotherapy and is recovering well. This report highlights an interesting case of ALC following COVID-19 infection presenting as an isolated facial rash. Whether there is a true relationship between the patient's COVID-19 infection and her abrupt presentation of leukemia remains unclear, but we present this case regardless, in an effort to highlight a potentially unique association requiring further study.

Pediatric leukemia cutis: A case series.

BACKGROUND: Pediatric leukemia cutis (LC) is often difficult to diagnose due to similarity in appearance to other dermatologic diseases. Several case reports and smaller case series have been published in the medical literature, but studies on larger cohorts of children with LC are lacking. OBJECTIVE: This study aimed to better characterize the clinical features, course, and prognosis of LC in the pediatric population. METHODS: We performed a retrospective case series of 31 patients diagnosed with LC at Boston Children's Hospital and the Children's Hospital of Philadelphia. RESULTS: The number and morphology of LC lesions varied among patients, with the head and lower extremities being the most common sites of involvement. Leukemia cutis presented concomitantly with systemic leukemia in the majority of cases. Most cases of LC arose during initial leukemia episodes, rather than with relapsed leukemia. Acute myeloid leukemia was the subtype most frequently associated with LC, followed by acute lymphoblastic leukemia. Diagnosis altered treatment timing and therapeutic decisions. CONCLUSION: Children most often present concomitantly with LC and systemic leukemia. Since the morphology and distribution of LC varies, physicians must maintain a high index of suspicion for this diagnosis, as the presence of LC may change the management of systemic leukemia.

An Unusual FDG-PET/CT Documentation in Aleukemic Leukemia Cutis: Atypical Penile, Scrotal, and Leg Muscles Involvement.

Aleukemic leukemia cutis (ALC) is a rare condition with a relatively poor prognosis, clinically presenting as skin and subcutaneous nodules commonly involving head and neck region with discrete atypical leukemic cells infiltrating in the skin on histopathology. The lesions on the genital area skin are seldom reported. A 49-year-old male presented with joint pains and multiple subcutaneous nodules clinically with biopsy proving it as leukemia cutis. Bone marrow aspiration and biopsy were suggestive of normocellular myeloid preponderant bone marrow with mild increase in eosinophils. The 18 F-fluorodeoxyglucose positron emission tomography/computed tomography ( 18 F-FDG PET/CT) revealed metabolically active FDG avid ill-defined soft tissue attenuation involving the entire length of penile shaft with central necrotic area, diffuse scrota skin thickening, and lateral compartment muscles of left leg, in addition to multiple hypermetabolic metastatic lymph nodes, and diffuse hypermetabolic marrow in humerii and femora. Despite prompt investigations and initiation of management, the patient succumbed in 15 days post-FDG PET/CT. The present report depicts an atypical case of ALC, with FDG PET-CT showing extensive and unusual sites of disease involvement, emphasizing its potential role in ALC.

Diagnostic challenge of aleukemic leukemia cutis preceding acute myelogenous leukemia: A case report.

Aleukemic leukemia cutis is a rare condition in which malignant white cells invade the skin before they appear in the peripheral blood or bone marrow. It is often associated with a poor prognosis. The condition presents a diagnostic challenge as its manifestations are quite variable terms of lesion type. It can manifest as papules, nodules, and/or plaques, and in rare cases erythematous macules, blisters, and ulcers. The most commonly affected areas of the body are the lower extremities, followed by the upper extremities, back, trunk, and face. Due to the non-specific presentation of the disease, skin biopsy and comprehensive immunohistochemical testing can be extremely helpful in the diagnostic work-up. We describe a case of leukemia cutis presenting prior to acute myelogenous leukemia that was initially misdiagnosed as hyper-IgG4 disease.

Multiple granulocytic sarcomas in a patient with longstanding complete remission of acute myelogenous leukemia.

Granulocytic sarcoma is an extramedullary tumor composed of granulocytic precursor cells. It usually presents as a nodular mass in the course of acute myelogenous leukemia. Rarely, the tumor develops in non-hematological conditions or in a patient with complete remission from the acute myelogenous leukemia. In such cases, aleukemic granulocytic sarcoma can be a preceding sign of systemic leukemia or a first sign of hematologic relapse of leukemia. We present an unusual case of multiple granulocytic sarcomas developed in a patient with longstanding complete remission of acute myelogenous leukemia, who has not had bone marrow and peripheral blood involvement for a long time.

Aleukemic leukemia cutis manifesting with disseminated nodular eruptions and a plaque preceding acute monocytic leukemia: a case report.

Aleukemic leukemia cutis (ALC), a discrete tumor of leukemic cells involving the skin, may be the first manifestation of acute myeloid leukemia, preceding the onset in marrow and blood by months and years. ALC is often difficult to diagnose and is associated with a dismal prognosis. A 63-year-old male presented with nodular swellings on the face, a plaque extending over the right shoulder and multiple enlarged cervical lymph nodes. The skin biopsy of the plaque lesion showed a diffuse neoplastic infiltration extending from the dermis to subcutaneous tissue with diffuse positivity for myeloperoxidase and focal positivity for CD34 on immunohistochemical staining. The diagnosis was leukemia cutis. One month later, acute monocytic leukemia (FAB AML-M5b) was diagnosed. The patient died on the seventh month of diagnosis.