Primary intraosseous hematopoietic pseudotumor: clinicopathological analysis and 9-year follow-up of 3 cases / 南方医科大学学报

We analyzed the clinicopathological data of 3 cases of primary intraosseous hematopoietic pseudotumor (IHPT), which had been previously misdiagnosed as malignancies or metastases both clinically and pathologically. Two of the patients received close follow-up for 132 and 100 months, and one patient was lost to follow-up, and the tumors were confirmed to be benign in all the 3 cases. IHPT is a rare benign intraosseous solid lesion consisting of tissues resembling normal hematopoietic tissue, and can be easily misdiagnosed as malignancy. Understanding the clinicopathological features and the outcomes of the disease can facilitate the clinical decisions on individualized diagnosis and therapeutic regimens.

Splenic myeloid metaplasia in warm autoimmune hemolytic anemia (wAIHA): a retrospective study

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.

Histomorphological Spectrum of Splenectomy Specimens in a Tertiary Teaching Hospital:A Seven Year Study

Background: Spleen is the largest organ of the mononuclear phagocytic system and is involved in all systemic inflammations, generalized hematopoietic disorders, and many metabolic disturbances. Splenectomy is frequently indicated where diagnosis is already established and the purpose of surgery being largely therapeutic in nature. Objectives: The study aims to highlight the histomorphological patterns in the surgically resected splenectomy specimens received in our centre and to correlate with the clinical indications. Materials and Methods: This was a seven year study of all splenectomy specimens received at histopathology section of Department of Pathology in a tertiary care hospital. All the histopathology slides were reviewed by the authors and the microscopic findings were studied and correlated with clinical data. Results: Twenty two (22) spleen samples were received during the study period with a M:F=1: 1.2 . The age range was 2 years to 60 years. The highest number of splenectomy cases was in the age group of 11-20 years followed by 0-10 years age group. The majority of the cases of splenectomy were indicated for thalassemia (14 cases, i.e. 63.64% of all cases), followed by traumatic rupture and secondary malignancy with 2 cases each (9.09% cases each), one case each for splenic infarct, myelofibrosis, ITP and splenic tuberculosis. Conclusion: Thalassemia was proved to be the commonest indication of splenectomy, followed by traumatic rupture and secondary malignancy in the spleen. Larger studies are needed for better understanding of this important organ and unnecessary splenectomies may thus be avoided

A Case of Extramedullary Hematopoiesis Associated with Congenital Dyserythropoietic Anemia / 소아외과

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.

Hematopoyesis extramedular renal: caso clínico / Renal extramedullary hematopoiesis: case report

Introducción: La Hematopoyesis Extramedular (HEM) se define como la producción de células precursoras de la línea eritrocítica, granulocítica y megacariocítica fuera de la médula ósea, con una rara incidencia, la cual se limita a reportes de casos. Objetivo: Describir un caso de HEM renal secundaria a un proceso hipóxico crónico. Caso clínico: Adolescente de 16 años portador de hipoxia crónica, cianosis central, acropaquia, viviendo a una altura superior a 2.500 metros sobre el nivel del mar. Hipoxia crónica, probablemente secundaria a Hipertensión Pulmonar, no pesquisada previamente ni tratada. Consultó por masa en hipocondrio izquierdo, de 3 meses de evolución. Ecocardiograma mostró Hipertensión pulmonar que no pudo ser valorada por estudios hemodinámicos. En la Tomografía Computarizada (TC) se evidenciaron masas intrarenales bilaterales, de predominio izquierdo e intrapélvicas. Hemograma con marcada policitemia, por lo que se realizó aspirado de médula ósea, que descartó patología maligna que la invada. La biopsia de la masa renal reportó HEM con sus tres líneas hematopoyéticas. Conclusión: Este caso de HEM, se plantea como respuesta al proceso de hipoxia crónica y no a lesión de medula ósea como usualmente se ha descrito.

Extramedullary hematopoiesis (EMH) is defined as the formation and development of erythrocytic, granulocytic and megakaryocytic cell lines outside the bone marrow; it is a rare occurrence, which is limited to case reports. Objective: To describe a case of renal EMH secondary to chronic hypoxia. Case report: The patient is a 16 year old teenager who developed chronic hypoxia, central cyanosis, clubbing and lives more than 2,500 m above sea level. Chronic hypoxia is probably secondary to pulmonary hypertension, neither detected nor previously treated. The Consultation was due to a mass in the left upper quadrant for the last three months. An Echocardiogram showed pulmonary hypertension that could not be assessed by hemodynamic parameters. A computed tomography showed bilateral left renal pelvic masses. The hemogram showed polycythemia, so bone marrow aspiration was performed, which rule out malignant pathology. A biopsy of the renal mass reported EMH with three hematopoietic cell lines. Conclusion: This EMH case is a response to chronic hypoxia rather than to a bone marrow lesion as it has usually been described.

Imaging diagnosis of extramedullary hematopoiesis / 实用放射学杂志

Objective To discuss and analyze the imaging features of extramedullary hematopoiesis (EMH).Methods Imaging data of EMH in 7 patients with 85 lesions confirmed by clinical and pathological results were retrospectively analyzed.Results CT or MRI showed thoracic paravertebral multiple tumor-like soft tissue masses in 6 patients accompanied with pleural mass in 1,with liv-er mass in 1 and spleen mass in 1,and the masses in the distal ileum and in front of the inferior vena cava in 1.Thoracic paraverte-bral and pleural masses presented isodensity on plain CT and moderate enhancement on post-contrast CT.Among the 6 liver mas-ses,CT showed uniform density in 5 and small area of necrosis in 1.The only one spleen mass also appeared inner necrosis and mul-tiple small calcifications.The mass in the ileum presented thickened bowel wall with moderate enhancement,meanwhile the mass in front of vena cava presented obvious edge enhancement.MRI in 2 patients showed the masses with low signal on T1 WI and high sig-nal on T2 WI.Conclusion EMH has certain specific imaging characteristics,which may be helpful for correct diagnosis of EMH when combined with the clinical data.

Amiloidosis primaria asociada a mielofibrosis idiopática crónica. una relación inusual / Primary amyloidosis associated to chronic idiopathic myelofibrosis. An unusual relationship

Introducción: las amiloidosis son entidades caracterizadas por la acumulación extracelular de material fibrilar en cantidad suficiente para comprometer las funciones vitales del órgano afectado. El componente fibrilar mayor determina el comportamiento y la clasificación. Presentación de caso: se presenta el caso de un paciente con amiloidosis primaria asociada a una mielofibrosis idiopática crónica con hematopoyesis extramedular esplénica. Se trata de un paciente masculino de 61 años que ingresa con el diagnóstico de una miocardiopatía restrictiva infiltrativa, alteraciones hematológicas, hepatoesplenomegalia y polineuropatía sensitivo-motora. El estudio mediante biopsia medular confirma el diagnóstico de amiloidosis, mientras que la citología por aspiración con aguja fina del bazo demuestra hematopoyesis extramedular y junto a los estudios hematológicos se concluye como una mielofibrosis idiopática crónica. Conclusiones: en la institución, los estudios de imagen e histopatológicos son fundamentales para confirmar la presencia de la amiloidosis. La asociación de amiloidosis primaria y mielofibrosis idiopática crónica fue casual, constituyendo el primer reporte en la literatura.

Introduction: amyloidosis is the term used for diseases caused by the extracellular deposition of protein fibrils in tissues and organs. These diseases are defined and classified by the biochemical nature of the protein in the fibril deposits. Clinical case: it is presented a clinical case of a patient with the diagnosis of primary amyloidosis and chronic idiopathic myelofibrosis with extramedullary hematopoiesis in the spleen. A 61-year male patient is admitted due to presenting infiltrative restrictive cardiomyopathy, hematologic alterations, hepatosplenomegalia and sensorimotor neuropathy. Amyloidosis is diagnosed through bone marrow biopsy, while fine-needle aspiration cytology of the spleen demonstrates extramedullary hematopoiesis, and along with hematologic studies, it is concluded as a chronic idiopathic myelofibrosis. Conclusions: in Universitary Hospital, imaging and histological studies are important to confirm the diagnosis of amyloidosis. The causality between primary amyloidosis and chronic idiopathic myelofibrosis there has not been reported in the literature.

Intracranial Extramedullary Hematopoiesis in Beta-Thalassemia

Extramedullary hematopoiesis (EMH) represents tumor-like proliferation of hemopoietic tissue which complicates chronic hemoglobinopathy. Intracranial EMH is an extremely rare occurrence. Magnetic resonance imaging (MRI) offers a precise diagnosis. It is essential to distinguish EMH from other extradural central nervous system tumors, because treatment and prognosis are totally different. Herein, we report the imaging findings of beta-thalassemia in a 13-year-old boy complaining of weakness of left side of the body and gait disturbance; CT and MRI revealed an extradural mass in the right temporoparietal region.

Cutaneous Extramedullary Hematopoiesis in Idiopathic Myelofibrosis / 대한피부과학회지

Extramedullary hematopoiesis is commonly seen in the liver, spleen and lymph nodes, but cutaneous extramedullary hematopoiesis (CEH) is very rare. CEH affects mainly children with intrauterine viral or hematologic disorders and rare manifestation of chronic myeloproliferative processes in adults. Idiopathic myelofibrosis (IM) is a chronic myeloproliferative disorder and some cases of IM have extramedullary hematopoiesis. IM is characterized by the proliferation of the endothelial cells and fibroblasts in the bone marrow, resulting in disruption of bone marrow, with subsequent migration and proliferation of the hematopoietic stem cells in other organs. We report a very rare case of cutaneous extramedullary hematopoiesis in a 74-year-old male with IM.

Osseous metaplasia and mature bone formation with extramedullary hematopoiesis in follicular adenoma of thyroid gland.

Follicular adenomas of the thyroid may be subjected to degenerative changes like hemorrhagic and cystic changes, fibrosis, and calcification. Mature bone formation is a rare phenomenon, but extramedullary hematopoiesis (EMH) has also been rarely reported in thyroid gland. The combination of mature bone formation and EMH is rarer and has been reported, in a single case report, in a multinodular goitre. We describe a case of follicular adenoma with histologically proven osseous metaplasia and mature bone formation with EMH in a middle- aged woman, which, to our knowledge, is the first case in English language literature.

A Case of Extramedullary Hematopoiesis Presenting as a Lung Mass in a Patient with Primary Myelofibrosis / 결핵및호흡기질환

Primary myelofibrosis is characterized by replacement of bone marrow with fibrotic tissue and the development of extramedullary hematopoiesis. Extramedullary hematopoiesis primarily involves the spleen and liver, but can also occur in the lungs. We report the case of an 80-year-old male who was admitted for evaluation of a lung mass and persistent thrombocytopenia. A percutaneous needle aspiration from the mass in the right lower lung showed myelopoietic cells with fatty tissue. A bone marrow biopsy revealed a hypercellular marrow with an increased number of atypical megakaryocytes. The final diagnosis was a prefibrotic stage of primary myelofibrosis leading to extramedullary hematopoiesis in the lung.

Intrahepatic Extramedullary Hematopoiesis Mimicking a Hypervascular Hepatic Neoplasm on Dynamic- and SPIO-Enhanced MRI

We present a rare case of a focal intrahepatic extramedullary hematopoiesis (EMH) that mimicked a hypervascular hepatic neoplasm in a 33-year-old woman with idiopathic myelofibrosis. The lesion showed homogeneous and persistent enhancement on both contrast-enhanced CT and gadolinium-enhanced dynamic MR imaging. The lesion did not demonstrate an apparent signal drop on a T2*-weighted sequence following administration of a superparamagnetic iron-oxide agent (SHU 555A). A hepatocellular adenoma was the initial radiological diagnosis. To the best of our knowledge, this is the first report of a histopathologically proven intrahepatic EMH evaluated with dynamic- and SPIO-enhanced MRI.

Low-dose, Whole-lung Radiotherapy for Pulmonary Extramedullary Hematompoiesis : A case report

A seventy-seven year old female was consulted for radiation therapy due to the infiltration of lung and pleural effusion from myelofibrosis. She was diagnosed clinically as extramedullary hematopoiesis (EMH), and had severe dyspnea and cardiac dysfunction originated from pulmonary hypertension. She underwent low-dose (2 Gy) whole-lung radiation therapy in 4fractions of 50 cGy each. Clinical symptom, especially dyspnea, and chest X-ray imaging was markedly improved after radiation therapy. We experienced that the low-dose whole-lung radiation therapy to EMH in lung might be effective for the palliation of pulmonary hypertension

Two Cases of Myelofibrosis Mimicking Malignant Lymphoma in Computed Tomography of Abdomen: A Case of Autoimmune Myelofibrosis associated with Systemic Lupus Erythematosus Showing Extensive Lymphadenopathy and A Case of Chronic Idiopathic Myelofibrosis wit / 대한진단검사의학회지

Myelofibrosis results from stimulation of bone marrow stromal fibroblasts by fibrogenic cytokines elaborated by neoplastic or reactive cells in the marrow. Chronic idiopathic myelofibrosis should be differentiated from secondary myelofibrosis resulting from bone marrow involvement of malignant lymphoma because these diseases have different therapeutic strategies. Myelofibrosis in systemic lupus erythematosus is an uncommon but well-recognized complication, and identifying an autoimmune myelofibrosis is important in diagnosing this benign cause of myelofibrosis. We report two cases of myelofibrosis presenting the clinical and radiologic findings that mimicked malignant lymphoma -a case of autoimmune myelofibrosis associated with systemic lupus erythematosus showing extensive lymphadenopathy and a case of chronic idiopathic myelofibrosis with focal intrasplenic extramedullary hematopoiesis- and discuss the importance of the clinical information and radiologic findings for the pathologic diagnosis of myelofibrosis.

A Case of Chronic Myelogenous Leukemia with Extramedullary Hematopoiesis Presenting as Liver Mass / 대한혈액학회지

Extramedullary hematopoiesis denotes blood cell production in hematopoietic tissue other than bone marrow. Myeloproliferative disorders, including myelofibrosis, polycythemia vera, essential thrombocythemia and chronic myelogenous leukemia (CML) are the most common cause of extramedullary hematopoiesis. Extramedullary hematopoiesis most commonly occurs in the spleen, liver and lymph nodes but has been reported in almost all sites of the body. Usually extramedullary hematopoiesis has been reported to involve liver, without forming a liver mass in a patient with CML. We report a patient with CML who had extramedullary hematopoiesis presenting as a liver mass established by ultrasound-guided biopsy.

Imaging Analysis of Intrathoracic Tumor-simulating Extramedullary Hematopoiesis / 实用放射学杂志

Objective To improve the accuracy of imaging diagnosis of intrathoracic tumor-simulating extramedullary hematopoiesis(EMH).Methods The chest radiographs and CT findings in two cases of intrathoracic tumor-simulating EMH with thalassemia were analyzed respectively and some related literatures were reviewed.Results A conventional chest X-ray showed rotundity or lobulated masses in the posterior inferior mediastinum in two cases, and CT scans showed smoothly marginated , homogeneous, lobulated paravertebral masses, on enhanced CT scans, the masses showed mild homogeneous enhancement.Conclusion Although intrathoracic tumor-simulating EMH is short of typical imaging features, the combination of clinical ,laboratory and radiological findings could make the diagnosis of intrathoracic tumor-simulating EMH, so unnecessary treatment or surgery could be avoided.

A Case of Juvenile Myelomonocytic Leukemia in a Six Months Old Infant / 대한혈액학회지

Myelodysplastic syndrome (MDS) in childhood is a rare hematologic malignancy and its classification has been the subject of some controversy. Cases of pediatric MDS are subdivided into those with features of adult-type MDS and those with myeloproliferative features occasionally observed in infancy and early childhood. There appears to be an international consensus to rename the disease juvenile myelomonocytic leukemia (JMML), which includes all leukemias of childhood previously classed as chronic myelomonocytic leukemia (CMML), juvenile chronic myelogenous leukemia (JCML), and infantile monosomy 7 syndrome. We experienced a 6-month-old female infant with JMML who developed extensive extramedullary hematopoiesis. The patient developed abdominal distention, hepatosplenome-galy, anemia, thrombocytopenia, and leukocytosis with significant monocytosis and was found to have a high hemoglobin F level of 30%. Her bone marrow biopsy section and aspirate smears revealed normocellularity with no increment of blast cells and no dysplastic changes. Cytogenetic analysis revealed a normal 46, XX karyotype. Her liver, spleen, lymph nodes, and appendix were found to be heavily infiltrated by partially differentiated myelomonocytic cells. These findings supported the diagnosis of JMML with extensive extramedullary hematopoiesis.

A Case of Cutaneous Extramedullary Hematopoiesis in Idiopathic Myelofibrosis / 대한혈액학회지

Idiopathic myelofibrosis is characterized by replacement of the bone marrow with fibrous tissue and the development of extramedullary hematopoiesis. The latter involves mainly the spleen and liver but also occurs in lymph nodes, kidneys, retroperitoneal fat, and more rarely, the skin. We report a 48-year-old male with idiopathic myelofibrosis who was admitted due to numerous papules and nodules on his trunk. The skin lesions were histologically defined as cutaneous extramedullary hematopoiesis.