Humans ; Hematopoiesis, Extramedullary ; Liver Neoplasms/diagnosis* ; Diagnostic Errors

Hematologic Diseases ; Hematopoiesis, Extramedullary ; Humans ; Mediastinal Neoplasms/surgery* ; Mediastinum ; Tomography, X-Ray Computed

Extramedullary hematopoiesis (EMH) is a rare cause of adrenal mass. We present a 44-year-old woman who has thalassaemia intermedia, referred to Endocrinology clinic for huge adrenal mass. Along with a paraspinal lesion discovered in this patient, the leading diagnosis was EMH. The patient was treated with hypertransfusion and hydroxyurea, which led to a reduction in the size of the right adrenal mass and paraspinal mass. This case highlights the challenges in managing this rare condition. Although EMH is a rare cause of adrenal mass, the diagnosis must be considered in any patient with a history of a congenital hemolytic disorder, to avoid unnecessary surgical procedures.
Hematopoiesis, Extramedullary

BACKGROUND: Splenic myeloid metaplasia (SMM) is a kind of extramedullary hematopoiesis, whereas its clinical significance in wAIHA remains unclear. The aim of this study is evaluating the frequency and clinical characteristics of SMM, compared with splenic-congestion (SC). METHODS: We included patients with wAIHA treated in a Mexican tertiary hospital between January 1992 and December 2015. All patients received steroids as first-line treatment and splenectomy as second-line treatment. RESULTS: Among the thirty-six splenectomized patients, 15 (41.6%) and 21 (58.4%) were diagnosed as SMM and SC, respectively. No differences were found in clinical characteristics between two groups. SMM patients showed lower platelet count (147×109/L vs. 240×109/L, P=0.02) and higher presence of anti-dsDNA antibodies (40% vs. 4.7%, P=0.01) than SC patients. Although the complete response (CR) rate with first-line treatment was lower in SMM patients (13.3% vs. 47.6%; P=0.04), post-splenectomy median disease-free-survival (DFS) was longer (16.2 mo vs. 5.1 mo; P=0.19). Univariate/multivariate analysis showed that achieving CR during first-line treatment (OR 0.3, 95% CI: 0.03–0.94, P=0.03) and higher platelet count (OR 0.99, 95% CI: 0.98–0.99, P=0.03) were protective factors for SMM; and anti-dsDNA titer higher than 9.6 IU/dL was a risk factor for SMM (OR 2.76, 95% CI: 1.48–5.14, P < 0.001). CONCLUSION: The wAIHA patients with SMM have different biological profiles with those without SMM. This study is the first trial evaluating the significance of histopathological spleen findings and their association with rheumatologic profile.
Anemia, Hemolytic, Autoimmune* ; Antibodies ; Hematopoiesis, Extramedullary ; Humans ; Platelet Count ; Primary Myelofibrosis* ; Protective Factors ; Retrospective Studies* ; Risk Factors ; Spleen ; Splenectomy ; Steroids ; Tertiary Care Centers

Hepatic perivascular epithelioid cell tumors (PEComas) are very rare. We report a primary hepatic PEComa with a review of the literature. A 56-year-old women presented with a nodular mass detected during the management of chronic renal failure and chronic hepatitis C. Diagnostic imaging studies suggested a nodular hepatocellular carcinoma in segment 5 of the liver. The patient underwent partial hepatectomy. A brown-colored expansile mass measuring 3.2×3.0 cm was relatively demarcated from the surrounding liver parenchyma. The tumor was mainly composed of epithelioid cells that were arranged in a trabecular growth pattern. Adipose tissue and thick-walled blood vessels were minimally identified. A small amount of extramedullary hematopoiesis was observed in the sinusoidal spaces between tumor cells. Tumor cells were diffusely immunoreactive for human melanoma black 45 (HMB45) and Melan A, focally immunoreactive for smooth muscle actin, but not for hepatocyte specific antigen (HSA).
Actins ; Adipose Tissue ; Blood Vessels ; Carcinoma, Hepatocellular ; Diagnostic Imaging ; Epithelioid Cells* ; Female ; Hematopoiesis, Extramedullary ; Hepatectomy ; Hepatitis C, Chronic ; Hepatocytes ; Humans ; Kidney Failure, Chronic ; Liver ; MART-1 Antigen ; Melanoma ; Middle Aged ; Muscle, Smooth ; Perivascular Epithelioid Cell Neoplasms

It has been known that extramedullary hematopoiesis occurring after birth can be developed in various diseases, and it is often found in hematologic diseases. Among these, congenital dyserythropoietic anemia is a rare disease characterized with increase of ineffective hematopoiesis and morphological abnormalities of erythroblasts. In congenital dyserythropoietic anemia, extramedullary hematopoiesis is very rare and only a few cases have been reported. Although treatment is not required if there is no symptom in extramedullary hematopoiesis, surgery or radiation therapy is effective in case that there is symptom or unresponsive anemia despite blood transfusion. This case report is about surgical treatment for extramedullary hematopoiesis in 23-year-old patients diagnosed of congenital dyserythropoietic anemia.
Anemia ; Anemia, Dyserythropoietic, Congenital* ; Blood Transfusion ; Erythroblasts ; Hematologic Diseases ; Hematopoiesis ; Hematopoiesis, Extramedullary* ; Humans ; Parturition ; Rare Diseases ; Young Adult

Plasmacytomas are extramedullary accumulations of plasma cells originating from soft tissue. Mediastinal plasmacytoma is a rare presentation. A 67-year-old man recovered after antibiotic treatment for community-acquired pneumonia. However, on convalescent chest radiography after 3 months, mass like lesion at the right lower lung field was newly detected. Follow-up chest computed tomography (CT) revealed an increase in the extent of the right posterior mediastinal mass that we had considered to be pneumonic consolidations on previous CT scans. Through percutaneous needle biopsy, we diagnosed IgG kappa type extramedullary plasmacytoma of the posterior mediastinum.
Aged ; Biopsy, Needle ; Diagnosis ; Follow-Up Studies ; Hematopoiesis, Extramedullary ; Humans ; Immunoglobulin G ; Lung ; Mediastinum ; Plasma Cells ; Plasmacytoma* ; Pneumonia* ; Radiography ; Thorax ; Tomography, X-Ray Computed

Extramedullary pulmonary hematopoiesis is a rare entity with a limited number of case reports in the available literature only. We report the case of a 66-year-old man with known primary myelofibrosis, in whom a Technetium-99m sulfur colloid bone marrow scan with single-photon emission computed tomography (SPECT)/CT revealed a pulmonary hematopoiesis as the cause of pulmonary hypertension and severe tricuspid regurgitation. To the best of our knowledge, this is the first description of Technetium-99m sulfur colloid SPECT/CT imaging in this rare condition.
Aged ; Bone Marrow/*radionuclide imaging ; *Hematopoiesis, Extramedullary ; Humans ; Hypertension, Pulmonary/*etiology/radionuclide imaging ; Lung/*radionuclide imaging ; Male ; Primary Myelofibrosis/complications ; Technetium Tc 99m Sulfur Colloid/diagnostic use ; Tomography, Emission-Computed, Single-Photon/methods ; Tomography, X-Ray Computed ; Tricuspid Valve Insufficiency/*etiology/radionuclide imaging

BACKGROUND: Myelofibrosis (MF) is a disease characterized by panmyelosis with intact maturation, progressive bone marrow fibrosis, and splenomegaly with extramedullary hematopoiesis. It is the most heterogeneous disease in the group of myeloproliferative neoplasms in terms of patient characteristics and natural history of the illness. Rationale and
OBJECTIVES: This study aims to describe the clinical, laboratory profile and survival of myelofibrosis patients seen in our institution and also to descriptively compare these characteristics of Filipino MF patients to Chinese and White MF patients.
RESEARCH DESIGN AND METHODOLOGY: An analysis of patients' data retrospectively collected through review of clinical records from 1994 to 2012 was done. All patients diagnosed with primary myelofibrosis (PMF) or post-polycythemia (post-PV) myelofibrosis or post-essential thrombocytosis (post-ET) myelofibrosis were included in this study. Survival was measured from first day of diagnosis to death from any cause or last known follow-up or direct communication to patient/relatives. Survival data were estimated using the Kaplan-Meier method. We compared the clinical characteristics and laboratory profile at diagnosis of these Filipino myelofibrosis patients to the Chinese as reported by Xu et. al., and White MF patients as reported by Cervantes et al.
RESULTS/SUMMARY: In our patients' data set, they seem to be younger with a median age of 57, less likely having constitutional symptoms but they have higher frequency of anemia, splenomegaly and leukocytosis. The heterogeneity of myelofibrosis' clinical and laboratory features were seen in this epidemiologic study on Filipino myelofibrosis patients in comparison with Chinese and White MF patients. The clinical course is a slowly progressive chronic disease with a median survival probability of 180 months.

Human ; Male ; Female ; Aged ; Middle Aged ; Adult ; Primary Myelofibrosis ; Splenomegaly ; Hematopoiesis, Extramedullary ; Leukocytosis ; Polycythemia ; Thrombocythemia, Essential ; Myeloproliferative Disorders ; Hematologic Diseases ; Anemia

Thoracic extramedullary hematopoiesis (EMH) is a rare disease entity that is usually associated with hematologic disorders, such as myelodysplastic or hemolytic disease. Because thoracic EMH is usually encountered as a mass during radiologic examinations, it should be differentiated from posterior mediastinal neurogenic tumors. Here, the authors report a case of EMH associated with hereditary spherocytosis. The patient underwent a complete excision by thoracoscopic surgery to differentiate it from other mediastinal tumors.
Hematopoiesis, Extramedullary ; Humans ; Mediastinal Neoplasms ; Mediastinum ; Rare Diseases ; Spherocytosis, Hereditary ; Thoracoscopy