Systemic mastocytosis with associated clonal hematologic non-mast cell lineage disease (SMAHNMD) is a subtype of systemic mastocytosis (SM) characterized by neoplastic proliferation of mast cells in association with a hematologic neoplasm defined by the World Health Organization (WHO) criteria. SM-AHNMD is more commonly associated with myeloid neoplasms such as, chronic myelomonocytic leukemia ...

The prognostic heterogeneity of the World Health Organization category of "systemic mastocytosis with associated clonal hematologic nonmast cell lineage disease" (SM-AHNMD) has not been systematically validated by primary data. Among 138 consecutive cases with SM-AHNMD, 123 (89%) had associated myeloid neoplasm: 55 (45%) myeloproliferative neoplasm (SM-MPN), 36 (29%) chronic myelomonocytic leuk...

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that possesses a heterogenous clinical and immunophenotypic presentation. The current case report describes an interesting and unique presentation of BPDCN as a primary paranasal sinus tumor without evidence of cutaneous or systemic involvement. As such, the report further contributes to the ongoing debate reg...

Blastic plasmacytoid dendritic cell neoplasm is an aggressive hematologic malignancy with a poor prognosis. No consensus regarding optimal treatment modalities is currently available. Targeting the nuclear factor-kappa B pathway is considered a promising approach since blastic plasmacytoid dendritic cell neoplasm has been reported to exhibit constitutive activation of this pathway. Moreover, nu...

Myasthenia gravis (MG) is a heterogeneous autoimmune disease, which characterized by postsynaptic neuromuscular transmission defect, with antibodies directly targeting the acetylcholine receptor (AChR) or other structural proteins of junction. The majority MG cases are associated thymic pathologies, including thymoma, thyroiditis, diseases malignant hematologic neoplasia. present study reported...

Simultaneous multilineage hematologic malignancies are uncommon and associated with poorer prognosis than single-lineage leukemia or lymphoma. Here, we describe a concomitant malignant neoplasm in 4-year-old boy. The child presented massive lymphoproliferative syndrome, nasal breathing difficulties, snoring. Morphological, immunocytochemical, flow cytometry diagnostics showed coexistence of acu...

Extramedullary hematopoiesis (EMH) occurs as a complication of hematologic disorders such as myelofibrosis, sickle cell anemia and thalassemia. The extramedullary tissue usually involves liver, spleen and lymph nodes, less frequently the chest. We present a recent case of a man with myeloproliferative neoplasm who developed pulmonary hemorrhage secondary to EMH in the lung and pulmonary artery....

Malignancies such as solid tumors and hematologic malignancies can often induce or be associated with Henoch-Schönlein purpura (HSP) in older males but not in children. Described here is the case of a 5-year-old boy who clinically presented with HSP. An imaging study of the abdomen revealed a right retroperitoneal neoplasm that histopathology postoperatively confirmed to be a neuroblastoma. Mal...

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy with aggressive clinical course and poor prognosis. Diagnosis is based on detection of CD4+ CD56+, CD123high, TCL-1+, and blood dendritic cell antigen-2/CD303+ blasts, together with the absence of lineage specific antigens on tumour cells. In this report we present a case of BPDCN presenting with extramedullar...