John R. Krause

Myeloid sarcoma as the presenting symptom of chronic myelogenous leukemia blast crisis.

Myeloid sarcoma is an extramedullary tumor composed of immature myeloid cells that efface the underlying tissue architecture. It is usually associated with acute myelogenous leukemia, but can be associated with myeloproliferative neoplasms, myelodysplastic disorders, or myeloproliferative/ myelodysplastic syndromes. If it is unrecognized, appropriate chemotherapy may be delayed and survival jeopardized. We present a case of a myeloid sarcoma presenting in an otherwise asymptomatic patient who ultimately was found to have chronic myelogenous leukemia, presenting in blast crisis. We also review the distinct clinical and pathologic features of myeloid sarcoma, as well as treatment of the disease.

Plasmablastic lymphoma following transplantation

Posttransplant lymphoproliferative disorder is a serious complication following solid organ as well as hematopoietic stem cell transplantation due to prolonged immunosuppressive therapy. Plasmablastic lymphoma, although classically associated with HIV infection, has since been described in transplant patients as a variant of posttransplant lymphoproliferative disorder with varying clinical presentations. Here we add two additional cases to the literature: one following lung transplantation and one following pancreatic transplantation. In addition, the demographic, therapeutic, and immunophenotypic characteristics from prior reported cases are summarized.

A Case Series of Clinically Undiagnosed Hematopoietic Neoplasms Discovered at Autopsy

OBJECTIVESnIn the United States, autopsy rates have diminished to less than 5% during the last half of the 20th century and the beginning of the 21st century for a multitude of reasons. Many believe this results in unrecognized malignancies that could have explained a patients death.nnnMETHODSnWe describe six deaths in which hematopoietic neoplasms were identified at autopsy but were not diagnosed clinically.nnnRESULTSnThe six undiagnosed hematopoietic malignancy cases discovered at autopsy include four men and two women ranging from 50 to 78 years of age. One patient was African American and five patients were white, all with multiple comorbidities. The tumors included diffuse large B-cell lymphoma, activated B-cell type, intravascular large B-cell lymphoma, ALK-negative anaplastic large cell lymphoma arising in a setting of human immunodeficiency virus, and a myeloid sarcoma.nnnCONCLUSIONSnThese cases illustrate the importance of the traditional postmortem examination in not only confirming clinical diagnoses but also identifying previously unknown diagnoses. Hematologic malignancies may present with nonspecific clinical manifestations, and this series of cases also emphasizes the necessity for widening the differential diagnosis in patients with unexplained lactic acidosis and hepatic failure to include hematopoietic malignancies since prompt treatment may be lifesaving.

An intravascular lymphoma with extravascular tendencies.

Intravascular large B-cell lymphoma (IVL) is rare and characterized by selective growth of neoplastic cells within the lumina of small blood vessels. We present the case of a 69-year-old woman who died of a widespread IVL with extravascular involvement of the lymph nodes, liver, bladder, and adrenal gland. This report discusses the unique features of IVL with concurrent extravascular components.

Adult T-cell leukemia/lymphoma.

Adult T-cell leukemia/lymphoma is a rare malignancy associated with the human retrovirus human T-cell lymphotropic virus type 1. It is characterized by the proliferation of highly pleomorphic lymphocytes. Involvement of peripheral blood, bone marrow, lymph nodes, spleen, and extranodal sites such as skin, liver, gastrointestinal tract, and central nervous system can occur. There are four distinct clinical variants, and the prognosis and clinical course range from highly aggressive to a more protracted course depending on the subtype. We describe a man with de novo adult T-cell leukemia/lymphoma and discuss the unique clinical, morphologic, immunophenotypic, and molecular features of this entity.

Hodgkin lymphoma transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

Transformation to a large cell lymphoma may occur during the course of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) in approximately 5% of the cases. This is known as Richters transformation. A much less frequent transformation to Hodgkin lymphoma may occur. We report a case of CLL/SLL in which a transformation to Hodgkin lymphoma occurred, and we review previously published reports of this transformation. Transformation to Hodgkin lymphoma in CLL/SLL has a poor outcome compared to de novo cases of Hodgkin lymphoma.

Plasmablastic Plasmacytoma of the Breast

Breast plasmacytomas are extremely rare entities that can be seen as primary malignant neoplasms in the absence of bone involvement or as secondary neoplasms from disseminated multiple myeloma. Clinicians should be aware of this entity, as it may mimic benign and malignant lesions in the breast. Microscopically, immature plasmacytomas may mimic other neoplasms, so caution should be made on histological examination to ensure the correct diagnosis and corresponding therapy. Here we present a case of a plasmablastic plasmacytoma of the breast in a 55-year-old woman that was originally thought to be an angiosarcoma.

A case of erythropoietic protoporphyria.

A 53-year-old Texas rancher developed a blistering skin rash that was sensitive to exposure to sunlight. He was referred to hematology with a presumptive diagnosis of porphyria. His peripheral blood counts were within normal limits, and a bone marrow examination revealed erythroid dyspoiesis and ringed sideroblasts. Serum, plasma, and erythrocyte protoporphyrin levels were elevated, the findings of which are consistent with a diagnosis of erythropoietic protoporphyria. This paper discusses the diagnosis and etiology of the porphyrias.

Primary follicular lymphoma of the duodenum

Follicular lymphoma, a common nodal lymphoma, is rare in the gastrointestinal tract. When seen in this location, duodenal involvement is frequent. Most patients have localized disease, and survival appears to be excellent even without treatment. Although the outcomes are improved, the morphologic, immunophenotypic, and genetic features remain similar to those of nodal follicular lymphomas. We describe a woman with de novo follicular lymphoma of the duodenum and discuss the features of this remarkably indolent variant of follicular lymphoma.

Simultaneous presentation of two distinct plasma cell neoplasms

Abstract Plasma cell neoplasms consist of a spectrum of diseases characterized by monoclonal proliferations of plasma cells. We report the simultaneous presentation of two distinct plasma cell neoplasms, a very uncommon situation.