Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm Occurring After Spontaneous Remission Of Acute Myeloid Leukemia: A Case Report And Review Of Literature
Xie, W., Zhao, Y., Cao, L., Huang, W., Wang, Y., & Huang, H. (2012). Cutaneous blastic plasmacytoid dendritic cell neoplasm occurring after spontaneous remission of acute myeloid leukemia: a case report and review of literature. Medical oncology (Norighthwood, London, England), 29(4), 2417–2422. https://doi.org/10.1007/s12032-012-0215-y
View Original Source →Abstract
Spontaneous remission of acute myeloid leukemia (AML) is an extremely uncommon event. The etiology is associated with infection, blood transfusion or granulocyte colony-stimulating factor therapy, which trigger immune responses to exert an antileukemic effect. The remission is usually temporary and followed by rapid relapse. However, we present a case of a 42-year-old man with spontaneous remission of AML-M5a, who did not relapse but developed a rare and aggressive lymphoma, named cutaneous blastic plasmacytoid dendritic cell neoplasm (BPDCN). The neoplasm cells are positive for CD4, CD56, CD43, CD45, and CD123, but negative for other lineage-specific markers. To our knowledge, this is the first report of BPDCN occurring after spontaneous remission of AML, although it has been observed that some BPDCN could shift to myeloid leukemia. Occurrence of the two diseases is more than a coincidence. Discovery of such cases may shed further light on the inner connection between BPDCN and myeloid disorders.
Case Details
Disease Location
Bone marrowithblood/skin
Personal Characteristics
42 -year-old male
Clinical Characteristics
Was admitted in september 2008 with a fever of 40c, repeated fatigue and dizziness blood routine showed pancytopenia biochem tests noted elevations in ldh, ferritin, and c reactive protein. Both urinary lambda and kappa light chains were elevated, with a normal kappa/lambda ratio chest CT scan showed consolidative patches in bilateral lower lobes and localized pleural thickening marrow aspirate was hypercellular, containing 91.5% large blasts cells, which had folded nuclei with fine chromatin and inconspicuous nucleoli blasts were weakly positive for peroxidase and sudan black, and strongly positive for non-specific esterase staining with inhibition of the reaction by naf, consistent with acute myeloid leukemia-m5a flow cytometry showed the blasts were positive for CD33, 64, 56, 65s, and 38 diagnosis for acute myeloid leukemia was made, patient also had an active pneumonia infection. Antibiotics were administered, g-csf was also adminned december 2010, patient presented with painless multicentric cutaneous lesions on the trunk, the lesions gradually increased in size and presented as dusky erythematous nodules of approx. 3cm in diameter skin biopsy revealed lymphoid infiltration of the dermis, consisting of medium-sized monomorphic blasts immunostaining showed the neoplastic cells positive for CD4, 56, 43, 45, and 123 patient was then diagnosed with CD4+/CD56+ blastic plasmacytoid dendritic cell neoplasm (bpdcn) flow cytometry of marrow showed 1.25% immature lymphocytes and were positive for CD20, 10, 19, sigm, and HLA-dr, indicating reactive proliferation of lymphocytes
Remission Characteristics
12 days after admission, hemoglobin unexpected begin to improve with gradually rising levels of leukocyte, concomitant with improvement in the clinical condition and resolution of fever a follow-up bone marrow study 2 weeks after the initial diagnosis of acute myeloid leukemia showed a hypocellular marrow with adequate megakaryocytes and absence of leukemic blasts re-evaluation by marrow aspiration 4 weeks later revealed normo-cellularity and remission of acute myeloid leukemia without blasts in the marrow flow cytometry of marrow showed that <0.07% of the gated monthsnuclear cells had similar cell surface markers previously demonstrated on the leukemic cells thus, complete remission following up for more than 24 months, clinical condiditon was stable and blood counts were normal patient received 2 cycles of chop and achieved complete remission of bpdcn
Treatment & Mechanisms
Proposed Remission Mechanisms
Sr may be due to cytokine release or cellular immune response during the course of pneumonia infection cytokines are known to increase in patients with severe infection, these cytokines are antileukemic. Also, severe febrile infections stiumlates NK cells and cytotoxic t lymphocytes, where are relevant for sr sr could be facilitated by g-csf therapy, g-csf can induce in vitro and in vivo differentiation of acute myeloid leukemia-m2 t(8; 21) and degradation of the acute myeloid leukemia1-eto oncoprotein g-csf could also suppress the leukemic cell clone by inducing apoptosis and suppressing the renewal of leukemic cells and induce the potential increase in effector cytotoxic cells
Clinical Treatment
Antibiotics were administered via IV starting with cefepime and continued with imipenem and fluconazole granulocyte colony-stimulating factor (g-csf) was administered to correcting leukopenia 2 cycles of chop
Non-Clinical Treatment
None reported