http://hemepathreview.com/UsefulHemeRefArticles/WHO%202008%20and%20Beyond.pdf

The 2008 WHO classification of lymphoid neoplasms and beyond: evolving concepts and practical applications.

Guidelines for the management of rheumatoid arthritis: 2002 Update

Rheumatoid arthritis (RA) is a progressive polyarthritis that is responsible for over nine million office visits annually. It is likely that most nurse practitioners will care for one or more patients with RA because approximately 1% of the adult population is affected by this disabling disorder. The guideline reviewed in this month’s column describes the recommended care of patients who have been previously diagnosed with RA. Author Grace Newsome, EdD, APRN-BC, FNP, is an Associate Professor of Nursing, North Georgia College & State University, Dahlonega, GA.

Guidelines for the management of rheumatoid arthritis: 2002 update - American College of Rheumatology Subcommittee on Rheumatoid Arthritis Guidelines

Diagnostic criteria and classification of mastocytosis: a consensus proposal

Rheumatoid arthritis (RA) is a progressive polyarthritis that is responsible for over nine million office visits annually. It is likely that most nurse practitioners will care for one or more patients with RA because approximately 1% of the adult population is affected by this disabling disorder. The guideline reviewed in this month's column describes the recommended care of patients who have been previously diagnosed with RA.

Guidelines for the Management of Rheumatoid Arthritis 2002 Update

Malignant fibrous histiocytoma, now termed high-grade undifferentiated pleomorphic sarcoma, is a term used to refer to a group of malignant soft-tissue tumors characterized by a storiform or cartwheel-like growth pattern [1–4]. This lesion is the most common malignant soft-tissue tumor of adult life, representing approximately 25% of all soft tissue sarcomas [5]. The undifferentiated pleomorphic sarcoma will demonstrate a wide range of histological appearances, and is often further subclassified, although in general large and deep lesions have a poorer prognosis [1]. Desmoid tumors are benign, locally aggressive, softtissue tumors that typically arise in association with fascia, aponeurosis and associated muscle. Often termed musculoaponeurotic fibromatosis, these non-metastasizing lesions show an infiltrative pattern of growth and a significant predilection for local recurrence [6, 7]. We report the extremely unusual association of an undifferentiated pleomorphic sarcoma arising within a desmoid tumor in a patient with Milroy’s disease (chronic hereditary lymphedema of unknown origin). To our knowledge, there have been no similar previous reports. Case report

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroy's disease.

Adrenal masses have varying presentations Most commonly, adrenal masses are discovered incidentally on CT or MRI during an evaluation for an unrelated complaint Although the majority of these are nonfunctional cortical adenomas, hormonally active tumors and adrenocortical carcinoma must also be considered in the differential diagnosis Rarely, retroperitoneal tumors may mimic an adrenal mass We report a case of a 49-year-old man with anemia and weight loss who was found to have a large retroperitoneal mass arising from the adrenal gland Surgical treatment involved en bloc resection of the right kidney, adrenal gland, segments 7 and 8 of the liver, and a portion of the right hemidiaphragm Final pathology revealed a low-grade myofibrosarcoma We believe that this is the first case report of a myofibrosarcoma of the adrenal gland Myofibrosarcomas are rare malignant tumors composed of myofibroblasts that arise from the deep soft tissues These tumors have a predilection for the head and neck, trunk, or extremities Myofibrosarcomas can be differentiated from other sarcomas by immunohistochemical staining and pathologic features We will briefly discuss the workup of an adrenal mass and focus on the diagnosis of myofibrosarcoma

Myofibrosarcoma of the adrenal gland.

Angiofollicular lymph node hyperplasia

Background Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of peripheral T-cell lymphoma accounting for less than 1% of non-Hodgkin lymphomas. It is generally associated with poor prognosis. Patients and Methods We performed a cohort study of patients with HSTCL treated at the Mayo Clinic between 1996 and 2020 exploring the clinical characteristics and therapeutic outcomes. Results Twenty-two cases of HSTCL were identified with a median (range) age at diagnosis of 45.5 (15.5-80.6) years and a male predominance (15/22, 68.2%). Clinical characteristics include massive splenomegaly in 16 patients (73%), hepatic involvement in 13 (59%), and chronic immunosuppressed state in 8 (36%). Phenotypically, lymphoma cells had gamma/delta T-cell receptor expression in 18 (82%) and alpha/beta in 4 patients. Cytogenetic abnormalities included isochromosome 7q (i7q) in 8 (62%) of 13 and trisomy 8 in 4 (44%) of 9. The median (range) follow-up of surviving patients was 33 (2.5-137) months. The median progression-free and overall survival were 9.5 months (95% CI, 1.8, 16.3) and 12.4 months (95% CI, 4.9, 18.5), respectively. Long-term survival was seen in 4 (18%) of 22 patients, with survival of 55, 74, 95, and 137 months. Moreover, 3 of 4 long-term survivors had splenectomy as part of initial treatment, and 2 of 4 long-term survivors received an allogeneic hematopoietic cell transplant (allo-HCT). Conclusion Liver involvement and chronic immunosuppression were associated with shorter survival. Although splenectomy and allo-HCT have anecdotal benefit in the literature, our data do not show a statistically significant benefit of splenectomy and/or allo-HCT, likely as a result of our small sample size.

Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience

Concurrent presentation of acute promyelocytic leukemia (APL) with other hematologic diseases in the absence of previous chemotherapy or ionizing radiotherapy treatment is very rare. We present a case of simultaneous occurrence of APL with myelodysplastic syndrome (MDS)-related acute myeloid leukemia (AML). A 43-yearold female presented with 3 month of history fatigue, night sweats, chills and pancytopenia. Bone marrow aspirate and biopsy demonstrated 20% myeloid blasts with dysplastic changes admixed with abnormal promyelocytes. Cytogenetic analysis showed tetraploidy and deletion in chromosomes 5q and 7q and polymerase chain reaction showed presence of PML/RARA mRNA transcripts, confirming the presence of concurrent APL and MDS-related AML. Induction chemotherapy with cytarabine and daunorubicin was initiated along with all-trans retinoic acid. This is the first case to be reported in the literature of concurrent occurrence of APL with MDS-related AML. Treatment with 7 + 3 regimen and ATRA was successful in inducing complete remission.

/pdf/myelodysplasia-related-acute-myeloid-leukemia-and-acute-2bejcjn1pc.pdf

David M. Menke

Papers

Soft-tissue malignant fibrous histiocytoma (high-grade undifferentiated pleomorphic sarcoma) arising in a desmoid tumor in a patient with Milroy's disease.

Myofibrosarcoma of the adrenal gland.

Angiofollicular lymph node hyperplasia

Outcomes of Hepatosplenic T-Cell Lymphoma: The Mayo Clinic Experience

Myelodysplasia-related acute myeloid leukemia and acute promyelocytic leukemia: concomitant occurrence of two molecularly distinct diseases.