ABSTRACT
OBJECTIVE@#To investigate the clinicopathological features of blastic plasmacytoid dendritic cell neoplasm (BPDCN).@*METHODS@#A total of 13 cases of BPDCN diagnosed in Peking University First Hospital from January 2013 to March 2022 were collected. The clinical features, histopathological characteristics, immunophenotypes and prognosis of the patients were analyzed retrospectively, and the related literatures was reviewed as well.@*RESULTS@#Among the 13 patients, 11 were male and 2 were female, with a median age of 62 years (ranging from 5 to 78 years). Among them, single organ involvement occurred in 5 cases, all of which presented with skin lesions. Two or more organs were involved in other 8 cases (single organ with bone marrow involved in 3 cases; skin, bone marrow and lymph node involved simultaneously in 3 cases; skin, bone marrow, lymph node and spleen involved simultaneously in 2 cases). Histopathologically, it was characterized by the proliferation of medium to large atypical blastic cells, which infiltrated the whole thickness of dermis. When involved, the bone marrow lesions mainly appeared in a diffuse pattern, while the lymph node structure was usually destroyed, and the red pulp of the affected spleen was diffusely invaded. Immunohistochemical staining showed that all the 13 cases were positive for CD4, CD56, and CD123 (13/13) in varying degrees. All the 9 cases expressed TCL1 (9/9). Variable expression of CD68 (KP1) (8/13), TdT (7/12), CD117 (2/6), and high Ki-67 proliferation index (40%~80%) were showed. The neoplastic cells lacked expressions of CD20, CD3, MPO, CD34, or CD30; EBER in situ hybridization were negative (0/9). After definite diagnosis, 6 cases received chemotherapy, among which 1 received adjuvant radiotherapy, and 2 received subsequent bone marrow transplantation. Another 2 cases only received maintenance treatment. The median follow-up time was 14 months (ranging from 6 to 36 months), 5 patients died of the disease (6 to 18 months), 3 patients survived (7 to 36 months up to now), and the remaining 5 patients lost follow-up.@*CONCLUSION@#BPDCN is a rare type of malignant lymphohematopoietic tumor with aggressive behavior and poor prognosis. The diagnosis should be made combining clinical features, histopathology, and immunohistochemical phenotype. Attention should be paid to differentiating BPDCN from other neoplasms with blastoid morphology or CD4+CD56+ tumors.
Subject(s)
Male , Female , Humans , Hematologic Neoplasms , Retrospective Studies , Dendritic Cells , Skin Neoplasms/pathology , Skin/pathologyABSTRACT
OBJECTIVE@#To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm(BPDCN).@*METHODS@#Clinical data of 5 patients diagnosed with BPDCN in Wuhan First Hospital and Wuhan Tongji Hospital from June 2016 to November 2021 were retrospectively analyzed.@*RESULTS@#Among the 5 patients, 3 were male and 2 were female, with a median age of 28(10-52) years old. Four patients showed obvious skin damage at the initial diagnosis; the other one showed clinical manifestations of acute leukemia rather than obvious skin damage at the initial diagnosis, but infiltrated skin when the disease relapsed after treatment. Other infiltration sites of lesions included bone marrow (2/5), peripheral blood (2/5), lymph nodes (3/5), liver and spleen (2/5). All patients had no clinical manifestation of central nervous system infiltration. Tumor cell specific immune markers CD4, CD56, CD123 were all positive, and the median Ki-67 index was 70%. TET2, ASXL1 and NRAS gene mutations were found respectively in 3 patients by next-generation sequencing technique (NGS). ALL-like, AML-like and invasive NK/T cell lymphoma-like first-line induction chemotherapy regimens were used for the patients. One patient died of severe complications during the early stage of chemotherapy, 3 patients were evaluated as CR, and 1 patient was evaluated as PR. 2 patients were recurred and progressed after induction of chemotherapy, and one of them was evaluated as CR after re-treatment. One patient received autologous hematopoietic stem cell transplantation (auto-HSCT) and got long-term survival (OS 87 months). 3 patients received allogeneic hematopoietic stem cell transplantation (allo-HSCT), of which one died of transplantation related complications, and 2 cases survived. The median follow-up time of 4 patients with evaluable efficacy was 28.5(9-84) months, the median OS time was 31.5(10-87) months.@*CONCLUSION@#BPDCN is a highly heterogeneous malignant tumor with a poor prognosis. HSCT, especially allo-HSCT can significantly improve the prognosis of BPDCN patients.
Subject(s)
Humans , Male , Female , Adult , Middle Aged , Retrospective Studies , Leukemia/pathology , Hematopoietic Stem Cell Transplantation , Prognosis , Myeloproliferative Disorders , Skin Neoplasms/pathology , Acute Disease , Dendritic CellsABSTRACT
OBJECTIVE@#To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).@*METHODS@#The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively.@*RESULTS@#4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine.@*CONCLUSION@#The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.
Subject(s)
Humans , Dendritic Cells , Retrospective Studies , Skin Neoplasms/pathology , Antineoplastic Agents/therapeutic use , Bone Marrow/pathology , Myeloproliferative Disorders , Hematologic Neoplasms/drug therapyABSTRACT
RESUMEN La neoplasia blástica de células dendríticas plasmocitoides (NBCDP) es una malignidad hematológica poco frecuente y generalmente agresiva, por lo cual se requiere su reconocimiento precoz. A continuación, se describe el curso clínico prolongado de un paciente masculino de 60 años con NBCDP procedente de Venezuela, en cuyos hallazgos más relevantes destacó la presencia de lesiones cutáneas, organomegalias, infiltración de la médula ósea y del sistema nervioso central. Posterior al diagnóstico se indicó quimioterapia sistémica, no obstante, el paciente falleció por complicaciones respiratorias durante la fase de inducción del tratamiento. En esta enfermedad es necesario establecer el diagnóstico diferencial con trastornos linfoproliferativos, leucemias linfoides y mieloides agudas, constituyendo el análisis morfológico de las células neoplásicas un aspecto importante para una adecuada orientación diagnóstica.
ABSTRACT Blastic plasmacytoid dendritic cell blast neoplasm (BPDCN) is a rare and generally aggressive hematologic malignancy, requiring early recognition. Below is a description of the prolonged clinical course of a 60-year-old male patient with BPDCN from Venezuela, whose most relevant findings highlighted the presence of skin lesions, organomegaly, infiltration of the bone marrow and central nervous system. Systemic chemotherapy was prescribed after diagnosis; however, the patient died of respiratory complications during the induction phase of treatment. In this disease, it is necessary to establish the differential diagnosis with lymphoproliferative disorders, acute lymphoid and myeloid leukemias. The morphological analysis of neoplastic cells is, thus, an important aspect toward proper diagnostic guidance.
Subject(s)
Humans , Male , Middle Aged , Skin Neoplasms/diagnosis , Dendritic Cells/pathology , Leukemia, Myeloid, Acute/diagnosis , Skin Neoplasms/pathology , Leukemia, Myeloid, Acute/pathology , Diagnosis, Differential , Lymphoproliferative Disorders/diagnosisABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a kind of highly malignant tumor in the hematological system, which is characterized with low incidence, diverse clinical manifestations, strong invasiveness and poor prognosis, however, currently there has no standard treatment yet. This paper reviews the recent research progress of BPDCN.
ABSTRACT
Introduction@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells. This malignancy presents with various noticeable cutaneous lesions and usually occurs in elderly males. Cutaneous manifestations usually precede leukemic dissemination to the lymph nodes, bone marrow, and peripheral blood which is associated with poor prognosis.@*Case presentation@#We present a case of a 60-year-old Filipino male with a four-month history of multiple hyperpigmented, reddish brown, firm, fixed, non-tender cutaneous nodules on the extremities, trunk, chest, and face. Two large masses was also noted on the left arm and left upper back..Tissue biopsy of the cutaneous mass showed Immunohistochemical stain findings positive for LCA, CD68, CD4, CD56, and CD123 which are compatible with BPDCN. Patient was initially asymptomatic with relatively normal blood count and was treated supportively but serial blood count monitoring showed worsening with progression to acute myelogenous leukemia. Patient was then started on the 7+3 protocol of cytarabine and idarubicine which provided flattening of the cutaneous nodules and improvement of blood counts. However, due to complications of the disease and the treatment, the patient succumbed to severe pulmonary infection and sepsis.@*Discussion@#Due to the varied, non-specific cutaneous manifestations and the similarity in the morphology of the skin lesions with other cutaneous conditions along with the rarity of this disease, there is difficulty in establishing the diagnosis of BPDCN as well as standardizing its treatment. Immunohistochemical stains play an important role in confirming the diagnosis as well as ruling out other differential diagnoses to tailor appropriate treatment.@*Conclusion@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) generally has a poor prognosis owing to the rapidity of its spread to the bone marrow and peripheral blood. Early diagnosis is essential to initiate early therapy and prevent progression.
Subject(s)
Leukemia, Myeloid, AcuteABSTRACT
@#Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematopoietic neoplasm with high malignancy and rapid progression. BPDCN is sensitive to chemotherapy, but it is prone to relapse and drug resistance, and the survival time is short. We report a case of BPDCN hospitalized in our department. In this case, the skin lesions were the first manifestation. The diagnosis of BPDCN was confirmed by the skin biopsy. Initially, this patient was sensitive to the lymphoma chemotherapy but the disease relapsed quickly, spreading to the lungs and mediastinum. Then, we treated the patient with myeloid regimen, which was no efficacy. The patient eventually died with an overall survival of 17 months.
ABSTRACT
Purpose To study the clinicopathological characteristics of blastic Plasmacytoid dendritic cell neoplasms, and improve the acknowledgement and diagnostic level. Methods The clinicopathological features, immunohistochemistry and EBV test results of 10 cases with BPDCN were analyzed retrospectively, with review of literature. Results Skin lesions were the first symptom in 10 patients, tumor cells expression of CD43, CD56, CD4 was positive in 8 cases, CD123 were positive in 5 cases, the T and B cell specific markers is not expression. EB virus encoded small RNA(EBER ) in situ hybridization is negative;no clonal rearrangement of B cell receptor(BCR) or T cell receptor(TCR) gene. Conclusion BPDCN is a rare malignant tumor of the skin, easy to involve the skin. It is necessary to combine clinical with pathological for definite diagnosis.
ABSTRACT
Objective To improve the understanding of blastic plasmacytoid dendric cell neoplasm (BPDCN). Methods The clinicopathological features of 2 cases with BPDCN diagnosed in the Department of Pathology in Cancer Hospital of Chinese Academy of Medical Sciences and Peking Union Medical College were analyzed retrospectively. The relevant literatures were reviewed as well. Results The two BPDCN patients were both elderly men with skin nodules. The tumor cells infiltrated diffusely into the whole dermis with sparing the epidermis. BPDCN cells were small to medium size with round or oval irregular nuclei and small nucleoli. Numerous mitoses figures were seen. Immunohistochemistry stain showed that CD4 and CD56 were positive and CD20 and CD3 were negative in the 2 BPDCN cases. Only 1 case was positive for CD123. Conclusions BPDCN is a rare malignant hematopoietic tumor, which is likely to involve skin. The features of morphology and immunophenotype are crucial to make correct diagnosis.
ABSTRACT
Objective@#To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm.@*Method@#Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed.@*Results@#Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) . In addition, extra-nodal involvement except skin was also observed, including breast (1/6) , maxillary sinus (1/6) , vertebrae (1/6) , and central nervous system (1/6) . Characteristic immunophenotype, CD4, CD56, and CD123 were all positive. All these patients were treated with acute lymphoblastic leukemia type (ALL-type) chemotherapy and complete remission (CR) were reached in 4 patients. The median follow-up was 9.5 (7-37) months, median progression free survival was 7 months; while median overall survival was 9 months. A total of 3 patients died during the follow-up, which were all happened in the first year after diagnosis, and all resulted from the relapse or disease progression.@*Conclusion@#Blastic plasmacytoid dendritic cell neoplasm is highly aggressive, in which the skin lesions are always manifested as initial symptoms, and bone marrow involvement, lymphadenectasis, splenomegaly, and hepatomegaly is also common. Characteristic immunophenotype include the positivity of CD4, CD56, and CD123. Effective and standard therapy is limited in this disease, which indicates the poor prognosis.
ABSTRACT
BACKGROUND/AIMS: Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that typically presents in the form of skin manifestations with or without lymph node and bone marrow involvement. Given its rarity and recent recognition as a distinct pathological entity, no standard of treatment exists for this aggressive disease and its prognosis is particularly dismal. METHODS: We retrospectively analyzed clinical features and treatment outcomes of patients who were diagnosed with BPDCN between 2000 and 2014. RESULTS: Ten patients had a median age at diagnosis of 41 years (range, 18 to 79), and seven patients were male. Sites of disease involvement were the skin (n = 7), lymph node (n = 5), bone marrow (n = 2), liver (n = 2), spleen (n = 2), and soft tissue (n = 1). Intensified chemotherapy regimens such as hyperCVAD regimen (cyclophosphamide, vincristine, doxorubicin, dexamethasone, methotrexate, cytarabine), and VPDL (vincristine, methylprednisolone, daunorubicin, L-asparaginase) were used as a first-line treatment. Although all patients treated with intensified chemotherapy showed an objective response (five patients with complete response) with median progression-free survival of 11.2 months (range 6.2 to 19.4), complete remission was not sustained for more than 2 years in any case. The response was relatively long-lived compared with previously reported CHOP (doxorubicin, cyclophosphamide, vincristine, prednisone)-like regimens, but the above regimens do not result in long-term remission. CONCLUSIONS: All patients treated with hyperCVAD or VPDL showed an objective response, but the duration of response was relatively short. Thus, the development of more effective induction as well as consolidation treatment strategy should be warranted to improve this rare disease entity.
Subject(s)
Humans , Male , Bone Marrow , Cyclophosphamide , Daunorubicin , Dendritic Cells , Dexamethasone , Diagnosis , Disease-Free Survival , Doxorubicin , Drug Therapy , Hematologic Neoplasms , Korea , Liver , Lymph Nodes , Methotrexate , Methylprednisolone , Prognosis , Rare Diseases , Retrospective Studies , Skin , Skin Manifestations , Spleen , VincristineABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, aggressive neoplasm classifi ed under “acute myeloid leukemia (AML) and related precursor neoplasm” by current WHO classifi cation. Elderly male are commonly affected with cutaneous lesion being the hallmark of disease presentation. The disease progresses rapidly and sooner or later involves bone marrow and peripheral blood. Cases presenting primarily as leukemia without cutaneous involvement is a rarity with about 29 cases reported in literature till date. Characteristic immunophenotype of CD4+/CD56+/− cells expressing antigens associated with plasmacytoid dendritic cells like CD123, TCL1, BDCA2/CD303, cutaneous lymphocyte-associated and interferon dependent molecule MxA, in absence of any other lineage specifi c marker confi rms the diagnosis. The disease has a poor survival and no standardized therapeutic strategy in the current scenario. A case of 25-year-male presenting with leukemic BPDCN without cutaneous involvement is presented here, who was treated with AML like protocol followed by hematopoietic stem cell transplantation, but succumbed to the disease within 8 months of diagnosis. The present case is being fi rst to be reported from India.
ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of acute leukemia that typically follows a highly aggressive clinical course in adults, whereas experience in children with this disease is very limited. We report cases of two children in whom bone marrow showed infi ltration by large atypical monocytoid ‘blast-like’ cells which on immunophenotyping expressed CD4, CD56, HLA-DR and CD33 while were negative for CD34 other T-cell, B-cell and myeloid markers. The differential diagnoses considered were AML, T/NKcell leukemia and acute undifferentiated leukemia. Additional markers CD303/ BDCA-2 and CD123 which are recently validated plasmacytoid dendritic cell markers were done which helped us clinch the diagnosis of this rare neoplasm. An accurate diagnosis of BPDCN is essential in order to provide prompt treatment. Due to its rarity and only recent recognition as a distinct clinicopathological entity, no standardized therapeutic approach has been established for BPDCN.
ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.
Subject(s)
Adolescent , Adult , Child , Humans , Male , Bone Marrow , Dendritic Cells , Diagnosis , Hematologic Neoplasms , Hematopoietic Stem Cell Transplantation , Induction Chemotherapy , Lymph Nodes , Precursor Cell Lymphoblastic Leukemia-Lymphoma , SkinABSTRACT
BACKGROUND: Blastic plasmacytoid dendritic cell neoplasm (BPDCN), which is derived from the precursor of plasmacytoid dendritic cells, is a rare and highly aggressive hematologic malignancy. It has only recently been recognized as a distinct entity. BPDCN characteristically has a predilection for cutaneous involvement. OBJECTIVE: The aim of this study was to describe the clinical and pathological features of BPDCN, and to review the treatment courses to analyze the prognosis and the optimal therapeutic approach. METHODS: We retrospectively reviewed seven BPDCN cases registered in the Samsung Medical Center database between January 2010 and December 2014. RESULTS: The median age of the patients was 52 years (range, 18~79 years), and six patients were male. The clinical staging was as follows: skin (n=5), lymph node (n=6), bone marrow (n=4), and peripheral blood (n=2). The skin manifestations were bruise-like tumefaction (n=4), erythematous nodule (n=4), or multiple erythematous papules (n=1). The pathological evaluation revealed dense diffuse or nodular infiltration of neoplastic cells, which were positive for CD4, CD56, and CD123 in the immunohistochemical analysis. Six patients received multiagent chemotherapy as the first-line treatment, alone (n=4), or followed by stem cell transplantation (SCT, n=1) or concurrent radiotherapy (n=1). The median progression-free survival after the first-line treatment was 6 months (range, 2~12 months). CONCLUSION: Three different skin manifestations were observed, with pathological features analogous to each other. All patients who received chemotherapy without SCT achieved partial or complete response but experienced relapse. Furthermore, they showed various clinical courses irrelevant to the cutaneous involvement.
Subject(s)
Humans , Male , Bone Marrow , Dendritic Cells , Disease-Free Survival , Drug Therapy , Hematologic Neoplasms , Lymph Nodes , Prognosis , Radiotherapy , Recurrence , Retrospective Studies , Skin , Skin Manifestations , Stem Cell Transplantation , Treatment OutcomeABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare hematological malignancy in children that is characterized by bruise-like skin lesions, with or without bone marrow involvement. Because the clinical course of BPDCN is highly aggressive and fatal in adults, allogeneic hematopoietic stem cell transplantation (HSCT) is recommended as the optimal treatment. Due to its rarity, the pediatric experience with BPDCN is limited and standard treatment has so far not been defined. We report a case of a 14-year-old boy with BPDCN involving the skin, bone marrow and lymph nodes. The patient was treated with high-risk group acute lymphoblastic leukemia protocol. He achieved a complete remission after induction chemotherapy and still maintains clinical remission without HSCT for 17 months after initial diagnosis.
Subject(s)
Adolescent , Adult , Child , Humans , Male , Bone Marrow , Dendritic Cells , Diagnosis , Hematologic Neoplasms , Hematopoietic Stem Cell Transplantation , Induction Chemotherapy , Lymph Nodes , Precursor Cell Lymphoblastic Leukemia-Lymphoma , SkinABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematopoietic malignancy which is derived from the precursors of plasmacytoid dendritic cells and is more infrequent in children than in adults. Formerly known as blastic NK-cell lymphoma or CD4+/CD56+ hematodermic neoplasm, the BPDCN is reclassified into the group of acute myeloid leukemia and related neoplasm by WHO in 2008. An 8-year old girl is being presented with bruise-like subcutaneous nodules with purpura on her right cheek from the performed biopsy. Histological examinations show sheet-like dense infiltrations of medium-sized lymphoid cells with irregular nuclei in the entire dermis. Immunohistochemical stainings of tumor cells were positive for CD4, CD56, LCA, TCL-1, TdT and focal positive for CD3, CD7, CD45RO and negative for CD20, CD30, CD34, EBV. The PET-CT scans indicate hot uptakes in the bone marrows which are suggestive of malignant infiltrations, and bone marrow biopsy findings are consistent with BPDCN of leukemic transformations. We present a rare case of BPDCN which affects the pediatric patient.
Subject(s)
Adult , Child , Female , Humans , Biopsy , Bone Marrow , Cheek , Dendritic Cells , Dermis , Hematologic Neoplasms , Herpesvirus 4, Human , Leukemia, Myeloid, Acute , Lymphocytes , Lymphoma , PurpuraABSTRACT
Objective To investigate the effect of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with intensified conditioning regimen followed by rapidly tapering immunosuppressants and sequential minimal residual disease (MRD)-guided donor lymphocyte infusion (DLI) post-transplantation on outcome of blastic plasmacytoid dendritic cell neoplasm (BPDCN).Methods Two cases of BPDCN from January 2009 to May 2011 in Nanfang hospital were diagnosed according to 2008 WHO classification of tumours of haematopoietic and lymphoid tissues.Case 1 initially presented with typical cutaneous involvement and was promptly diagnosed with CD+4CD+56LCA+TdT+CD+43 BPDCN by skin biopsy.Case 2 was recognized as acute lymphocyte leukemia and acute non-lymphocytic leukemia,which was diagnosed to BPDCN at recurrence through flow cytometry analysis.Total-body-irradiation plus cyclophosphamide based intensified conditioning regimen were followed by allo-HSCT from sibling donor.Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate.Anti-thymocyteglobulin was included additionally for haploid donor allo-HSCT.Multi-color labeling flow cytometry was performed to monitor MRD.Rapidly tapering of prophylactic immunosuppressants and sequential MRD-guided donor lymphocyte infusion (DLI) were performed to control relapse of primary malignancy.Results Two cases of BPDCN received allo-HSCT from sibling donor after intensified conditioning regimen.Both patients achieved complete remission and complete donor engraftment.Case 1 survived refractory acyclovir-resistant Epstein-Barr virus viremia benefiting from preemptive treatment with rituximab and DLI-induced grade Ⅳ acute GVHD,but died of thrombotic microangiopathy mixed with diffuse alveolar hemorrhage and sepsis on +243 days.Case 2 relapsed just 2 months after allo-HSCT despite DLI and rapidly tapering of CsA,died of sepsis followed by diffuse intravascular coagulation on +101 days.Conclusion BPDCN is characterized with typical cutaneous and/or bone marrow involvement with CD4+CD+56CD+123CD+43 blastic plasmacytoid dendritic cell and highly aggressive clinical course.Allo-HSCT seems to be a promising treatment for early phase of aggressive BPDCN aided with MRD monitoring and DLI,but it deserves more intensive researches to promote outcome of advanced staged BPDCN.
ABSTRACT
Los carcinomas basocelulares gigantes (CBCG) son muy poco frecuentes. Se los puede definir como aquellos mayores de 5 cm de diámetro. La mayoría de estos pacientes no refiere antecedentes deexposición crónica al sol ni presentan otros factores de riesgo conocidos. Existe en cambio una relación estrecha con situaciones de negligencia y tratamientos inadecuados. Se los encuentra frecuentemente en el tronco. Las lesiones mayores de 10 cm de diámetro tienen un alto riesgo de generar metástasis y en consecuencia producir la muerte. Presentamos una paciente de 92 años con dos masas tumorales en área malar y mejilla derecha que fue tratada quirúrgicamente, con buena evolución hasta los dos años de seguimiento posterior.
CD4+ / CD56 malignancy is an extremely rare hematological neoplasm, which was recentlyshown to correspond to the so-called type 2 dendritic cell or plasmacytoid dendritic cell. Clinicalpresentation typically correspond to cutaneous nodules o tumors associated with lymphadenopathyor spleen enlargement or both, cytopenias and circulating malignant cells. The prognosis is rapidlyfatal in the absence of chemotherapy. Bone marrow transplantation is the best option available. Wepresent two new cases of this disease.