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Plasma cell leukemia (PCL) is a rare variant of leukemia with an aggressive clinical course and a poor prognosis. The cutaneous involvement in PCL is very rare either at clinical presentation of leukemia, namely “leukemia cutis”, or in the metastatic PCL to the skin. We present a case of eruptive multiple cutaneous nodules in a 56-year-old man with metastatic PCL. Histologically, a diffuse dermal and subcutaneous infiltration of ovoid cells with amphophilic cytoplasm and eccentrically located nucleus consistent with plasmacytoid morphology was observed. Neoplastic cells showed strong immunoexpression for CD138 and CD38 consistent with plasma cells phenotype, and loss of expression of CD56. Kappa light chain restriction similar to the phenotype of his PCL was demonstrated. We suggest that the evaluation of new skin lesions in leukemic patients should include a histopathologic examination to establish the diagnosis as soon as possible and a correct management of the disease.

Abstract Introduction: Aleukemic leukemia cutis (ALC) is a rare condition and concerns less than 10% of leukemia cutis (LC) cases. LC is defined as a cutaneous infiltration of neoplastic myeloid or lymphoid blasts, which occurs in the absence of any prior bone marrow or peripheral blood involvement. Case Presentation: A pediatric case of B-cell ALL presenting as ALC is presented because of an exceptional testicular localization. Conclusion: B-cell acute lymphoblastic leukemia presenting as ALC is rarely described in the literature, and this case could be the first of childhood ALC with testicular involvement. Introduction: Aleukemic leukemia cutis (ALC) is a rare condition and concerns less than 10% of leukemia cutis (LC) cases. LC is defined as a cutaneous infiltration of neoplastic myeloid or lymphoid blasts, which occurs in the absence of any prior bone marrow or peripheral blood involvement. Case Presentation: A pediatric case of B-cell ALL presenting as ALC is presented because of an exceptional testicular localization. Conclusion: B-cell acute lymphoblastic leukemia presenting as ALC is rarely described in the literature, and this case could be the first of childhood ALC with testicular involvement.

Background In childhood acute lymphocytic leukemia (ALL), relapse is most commonly seen in the bone marrow (10–20%), followed by the central nervous system (3–8%). Isolated skin relapse is very rare in ALL. We report an 8-year-old child presented with isolated skin relapse. Case presentation An eight-year-old female patient presented with swelling on the scalp 3 months after the completion of the ALLIC-BFM 2009 chemotherapy protocol administered due to the diagnosis of precursor B-cell (pre-B) ALL. Physical examination revealed a hard, painless, hyperemic, nodule-shaped lesion measuring 2 × 1 cm on the right parietal bone. Atypical hematopoietic cells with the prominent nucleolus, narrow cytoplasm, and immunohistochemically stained with CD 10, 19, 22, 79-a, and TdT were observed in the histopathological examination of the skin lesion. There was no blast in the bone marrow aspiration smear and cerebrospinal fluid. The patient was diagnosed with aleukemic leukemia cutis (LC) and pre-B ALL, presenting as an isolated relapse. Conclusion Aleukemic LC is a very rare finding after leukemia treatment. It may present with various cutaneous lesions, such as a papule, macule, plaque, nodule, palpable purpura, and ulcerative lesions. Leukemia cutis should be considered in the differential diagnosis of skin lesions developing during or after treatment in children with leukemia.

Abstract Objectives Leukemia cutis is a conflicting term to describe neoplastic hematopoietic infiltrates in the skin. Cutaneous myeloid or lymphoid proliferations often present a serious differential diagnostic challenge for pathologists. Methods This review aims to outline the confusion associated with the term leukemia cutis and discuss in detail the foremost common differential diagnoses in daily practice. The review is based on a summary of the relevant literature as well as on the authors’ experience. Results It addresses precursor cell myeloid and lymphoid tumors that are strictly considered true leukemia cutis but also more mature neoplasms, including some recently described mature extramedullary myeloid proliferations. Finally, a practical, comprehensive stepwise approach combining traditional immunohistochemical marker panels, novel lineage- or mutational-specific markers, and other ancillary tests is suggested to reach an entity-specific diagnosis. Conclusion The proper combination of ancillary techniques can help the pathologist to provide an accurate diagnosis of these challenging skin lesions.

Background Extramedullary manifestations (EM) are rare in acute myeloid leukemia (AML) and their impact on clinical outcomes is controversially discussed. Methods We retrospectively analyzed a large multi-center cohort of 1583 newly diagnosed AML patients, of whom 225 (14.21%) had EM. Results AML patients with EM presented with significantly higher counts of white blood cells ( p  < 0.0001), peripheral blood blasts ( p  < 0.0001), bone marrow blasts ( p  = 0.019), and LDH ( p  < 0.0001). Regarding molecular genetics, EM AML was associated with mutations of NPM1 (OR: 1.66, p  < 0.001), FLT3 -ITD (OR: 1.72, p  < 0.001) and PTPN11 (OR: 2.46, p  < 0.001). With regard to clinical outcomes, EM AML patients were less likely to achieve complete remissions (OR: 0.62, p  = 0.004), and had a higher early death rate (OR: 2.23, p  = 0.003). Multivariable analysis revealed EM as an independent risk factor for reduced overall survival (hazard ratio [HR]: 1.43, p  < 0.001), however, for patients who received allogeneic hematopoietic cell transplantation (HCT) survival did not differ. For patients bearing EM AML, multivariable analysis unveiled mutated TP53 and IKZF1 as independent risk factors for reduced event-free (HR: 4.45, p  < 0.001, and HR: 2.05, p  = 0.044, respectively) and overall survival (HR: 2.48, p  = 0.026, and HR: 2.63, p  = 0.008, respectively). Conclusion Our analysis represents one of the largest cohorts of EM AML and establishes key molecular markers linked to EM, providing new evidence that EM is associated with adverse risk in AML and may warrant allogeneic HCT in eligible patients with EM.

Occult hematologic malignancies can initially present with cutaneous manifestations. We describe a 53-year-old woman with chronic hepatitis C and intravenous drug use presenting with a painful, progressive leg rash and worsening bicytopenias. A skin punch biopsy revealed neutrophilic dermatosis morphologically compatible with Sweet syndrome (SS), along with rare CD117+/CD34 − blastoid cells. Subsequent bone marrow studies revealed acute myeloid leukemia with mutated NPM1 . This case highlights the diagnostic challenge of distinguishing SS from leukemia cutis (LC) when skin biopsies demonstrate mixed neutrophilic with rare blastoid cells. LC represents cutaneous infiltration of leukemic blasts, whereas SS represents a non-neoplastic, cytokine-driven process. Prompt immunophenotypic evaluation and genomic testing of myeloid dermatoses, particularly those with blastoid cells, can be critical in distinguishing SS from LC to provide significant insight on prognosis and therapeutic options. This case highlights the clinical and histopathologic spectrum of myeloid dermatoses and further emphasizes the relevance of skin biopsies in the diagnosis of cutaneous involvement by hematologic malignancies, such as myeloid neoplasms.

Background The involvement of CCL2/CCR2 in leukemic cell infiltration into the skin, specifically in leukemia cutis (LC), remains largely unexplored. Oxidative stress has been demonstrated to induce the upregulation of CCL2 expression, while the antioxidant properties of azelaic acid (AZA) have been proven to exert anti-acute myeloid leukemia (AML) effects. Aims To investigate the role of CCL2/CCR2 in the pathogenesis of leukemia cutis, and to evaluate whether azelaic acid (AZA) can inhibit the infiltration of leukemic cells into the skin and its possible mechanism. Methods The migratory capacity of leukemic cells toward skin tissue was evaluated in vitro to determine the effects of CCL2/CCR2 and its modulation by AZA treatment. Concurrently, the regulatory effects of AZA on the NF-κB/MAPK signaling pathway in keratinocytes were investigated. In vivo studies were conducted using a patient-derived xenograft (PDX) AML mouse model, wherein AZA was administered via tail vein injection. A quantitative assessment of leukemic cell infiltration in skin tissues was performed to compare the therapeutic efficacy of AZA treatment versus untreated controls. Results AZA treatment significantly reduced CCL2 secretion in keratinocytes and decreased CCR2 expression in AML cells, consequently attenuating the migratory capacity of AML cells toward keratinocytes. Mechanistically, AZA downregulated the NF-κB/MAPK signaling pathway in keratinocytes. In vivo studies using the PDX-AML mouse model demonstrated that AZA administration markedly reduced leukemic cell infiltration in skin tissues compared to untreated controls. Conclusions CCL2/CCR2 plays an important role in the pathogenesis of LC. AZA effectively inhibits leukemic cell infiltration into the skin by downregulating CCL2 production through negative regulation of the NF-κB/MAPK signaling pathway in keratinocytes.

Isolated Cutaneous Myeloid Sarcoma (icMS) and Aleukemic Leukemia Cutis (ALC) are cutaneous extramedullary manifestations of leukemia in which leukemic cells infiltrate the skin before they can be identified either in the peripheral blood or in the bone marrow. We report the case of a 67-year-old patient who presented with a rapidly developing cutaneous tumour and scaly, erythematous-squamous plaques. Isolated Cutaneous Myeloid Sarcoma was diagnosed which rapidly progressed to terminal-stage Acute Myeloid Leukemia (AML). To highlight the disease characteristics of the adult-onset icMS and ALC cases that preceded AML we additionally compiled the pertinent literature of case reports of these rare conditions. We identified n=15 previously published icMS/ALC cases with adequately detailed clinical data descriptions. We could confirm the medical experience that icMS/ALC patients have an overall worse prognosis. Moreover, we could identify FAB M5 AML subtype as a significant adverse prognosticator of these patients.

Skin lesions have been reported in about 10–12% of hairy cell leukemia (HCL) patients. Most are etiologically related to autoimmune or infectious processes, although secondary cutaneous neoplasms and drug-induced lesions are also reported. However, leukemia cutis with the direct infiltration of the skin by leukemic cells is extremely rare in HCL patients. This paper reviews the epidemiology, pathogenesis, clinical symptoms, diagnosis, and approach to treating skin lesions in HCL. A literature review of the MEDLINE database for articles in English concerning hairy cell leukemia, skin lesions, leukemia cutis, adverse events, infectious, cutaneous, drug reactions, neutrophilic dermatoses, secondary neoplasms, and vasculitis was conducted via PubMed. Publications from January 1980 to September 2020 were scrutinized. Additional relevant publications were obtained by reviewing the references from the chosen articles.

The author describes paediatric case of relapsed acute lymphoblastic leukaemia (ALL) presented as aleukemic leukaemia cutis (ALC). A 2 year old child was admitted in tertiary oncology centre. He suffered from pre B cell ALL with absent Philadelphia chromosome. This patient received multiagent induction chemotherapy as per Berlin–Frankfurt–Munster (BFM) protocol for ALL. He achieved remission after 28 days of treatment. Subsequently he presented with multiple skin lesions in the form of multiple small erythematous violaceous macules, papules, plaques and nodules on face, chest and back regions. Histopathological examination of biopsy of skin revealed diffuse infiltration of tumor cells with prominent nucleoli, scant eosinophilic cytoplasm and numerous mitotic figures consistent with LC. Immunohistochemistry was positive for CD 10, CD 19, CD 22, CD 24, CD 79-a and TdT while negative for surface immunoglobulin. At the time of presentation his peripheral blood smear and bone marrow examination was negative for malignant cells. Sanctuary sites including central nervous system and testicles were not involved. So patient was diagnosed as ALC. He was managed as per BFM relapse protocol for ALL. Skin lesions disappeared completely after 2 weeks of treatment. Unfortunately patient developed bone marrow and testicular relapse after 2 months. He was given testicular radiotherapy and systemic chemotherapy for relapsed ALL. But his marrow was showing persistent activity and he expired after 4 months.