Diagnosis Of Extramedullary Hematopoiesis Research Articles

Extramedullary Hematopoiesis Identified in Pleural Fluid of a Patient with Bilateral Pleural Effusions and Associated Myelofibrosis

Abstract Introduction/Objective Extramedullary hematopoiesis (EMH) is the formation of blood cells outside of the bone marrow medullary spaces. EMH is typically seen in the spleen or liver and in association with bone marrow replacement disorders, hemoglobinopathies, and other hematological disorders. Rarely EMH can occur in the pleural cavity of patients resulting in subsequent pleural effusions. These cases can accurately be diagnosed with identification of megakaryocytes and other hematopoietic elements in the pleural fluid. Methods/Case Report We present a 67 year old male with a past medical history of pericardial effusion, Jak 2 positive Myelofibrosis (Post PV/ET), and type II diabetes mellitus, who presented with complaint of shortness of breath for 10 days. The patient did not require oxygen at home and denied any cough or hemoptysis. In the emergency department a Chest X ray was ordered which showed bilateral pulmonary effusions. These effusions were attributed to worsening congestive heart failure, and diuretic therapy was started. With no resolution of the pleural effusions, the decision was made to conduct a thoracentesis and remove the fluid. Interventional radiology then performed a thoracentesis removing 1000 mL from each the left and right pleural spaces. Cytological preparation of the fluid demonstrated myeloid and erythroid precursors as well as occasional megakaryocytes. The diagnosis of extramedullary hematopoiesis was made and the effusion did not recur. Results (if a Case Study enter NA) NA. Conclusion Extramedullary hematopoiesis of the pleural space is rare. However it must be considered in the differential diagnosis of a patient with persistent effusions and concomitant bone marrow replacement disease such as myelofibrosis. Prompt removal and cytological evaluation of the pleura fluid for hematopoietic elements should be performed in order to diagnose and manage the patient most effectively.

English

A 49-year-old female patient was admitted in the medicine department of Assam Medical College with complaints of paraparesis of 15 days duration and backache of 3 months’ duration. She had history of dizziness and low blood pressure. On general examination, she had tachycardia (heart rate of 126 beats / min), tachypnoea (respiratory rate 24 / min), severe pallor and bilateral non-pitting pedal oedema. The patient had low haemoglobin (6.6 gm / dl) with red blood cell (RBC) count of 2.5 million / mm3 . She was sent to the radio-diagnosis department for non-contrast computed tomography (CT) scan of thorax and contrast enhanced magnetic resonance (CEMR) of dorsolumbar region. On CT scan, soft tissue density lesions were noted in paraspinal region extending from lower thoracic, lumbar region without destruction of vertebral bodies. Additional findings include hepato-splenomegaly and left sided pleural effusion. On CEMR, there was T1 hypo and T2 / T2FSisointense soft tissue lesion in paraspinal region extending from lower thoracic, lumbar and sacral vertebrae. The lesions were showing diffusion restriction, diffusion weighted imaging (DWI) with low apparent diffusion coefficient (ADC) value and mild enhancement on post contrast scan. The soft tissue component was noted to extend into the epidural space in multiple vertebral levels in thoracic, lumbar and sacral regions causing spinal canal stenosis, maximum (7.0 mm) at L4 vertebral level. Based on the imaging findings, a diagnosis of extramedullary haematopoiesis in paraspinal region with extension of the soft tissue in the epidural space leading to compression of spinal cord at multiple levels was made.

Extramedullary Hematopoiesis in Adrenal Incidentaloma: Case Report of an Atypical Histopathological Finding

Adrenal gland extramedullary hematopoiesis is extremely rare, and when present, imaging studies may mimic an incidentaloma suspicious of malignancy. We present a 55-year-old woman without a history of hematologic disorder or neoplasms. There was an incidental finding of a 3.2 × 2.4-cm heterogenous adrenal mass and enhanced by contrast abdominal CT scan. Suspicious of malignancy was established, and laparoscopic left adrenalectomy was performed. Histopathological examination and immunohistochemical staining exhibited myeloid and erythroid precursors, as well as megakaryocyte confirming the diagnosis of extramedullary hematopoiesis in the adrenal gland.

Extramedullary hematopoiesis: A report of two cases

Extramedullary hematopoiesis (EMH) is defined as hematopoiesis occurring in organs outside of the bone marrow. The present report describes two cases of thalassemic patients with paraspinal medullary hematopoiesis and analyzes the clinical manifestations, imaging, pathology, diagnosis and treatment of EMH. In addition, a supplementary review of previously published cases is provided along with a review of the related literature. Computed tomography (CT) of the first case revealed multiple paraspinal masses, and the largest was 6.2×8.0 cm in diameter. Likewise, CT of the second patient revealed multiple paraspinal masses in the bottom of the left thoracic cavity, and the largest was measured 10.1×10.5 cm. The two cases underwent surgical biopsy and the findings were compatible with a diagnosis of EMH. In conclusion, EMH is a compatible and rare disease, and should be distinguished from other neoplasms. EMH must considered when masses with characteristic radiologic appearance are detected in patients with thalassemia intermedia.

Extramedullary Hematopoiesis in a Man With β-Thalassemia: An Uncommon Cause of an Adrenal Mass

Introduction Extramedullary hematopoiesis (EMH) commonly occurs in the spleen, liver and lymph nodes. Rare cases of EMH in the adrenal gland have been reported. Case Presentation We report the case of a 33-year-old man from the South of Iran suffering from major β-thalassemia, who underwent open left adrenalectomy and the histopathology revealed EMH. Conclusions In patients in which a history of hematologic disorders exists, careful imaging and hormonal assay should be done to certify a diagnosis of EMH. However, the surgical management becomes inevitable in certain cases.

A rare case of reversible paraplegia due to extramedullary hematopoiesis in a patient with thalassemia intermedia

30 yrs old man is known to have thalassemia intermedia presented with bilateral lower limb weakness and sphincteric troubles with loss of deep sensations, the motor power of the muscles and precipitancy of urine improved after blood transfusion, laboratory investigations and MRI dorsolumbar spine confirm the diagnosis of extramedullary haematopoiesis.

Bilateral Pleural Effusion in a Patient with an Extensive Extramedullary Hematopoietic Mass

We present a 56-year-old woman with bilateral pleural effusions, widespread enlarged lymph nodes, and soft tissue masses located within the renal pelvis. The initially working diagnosis was tuberculosis and lymphoma. Further pathological examination of the lymph node biopsy confirmed a diagnosis of extramedullary hematopoiesis, and a bone marrow biopsy revealed myelofibrosis. Unlike common treatment options such as radiotherapy and/or surgery, intrathoracic cisplatin and dexamethasone for the treatment of pleural effusions secondary to extramedullary hematopoiesis demonstrated an improvement in feasibility and efficacy in the present case.

Adrenal Extramedullary Hematopoiesis Associated with β-Thalassemia Major

The presence of apparently normal hematopoietic tissue outside of bone marrow cavity is defined as extramedullary hematopoiesis (EMH). EMH is a rare complication in thalassemia major (TM) and adrenal gland as well. This report describes a case of adrenal EMH in a 26-year-old man with β-TM. He has been transfused with regular blood transfusion since 9 months. During the routine physical examination he was incidentally found to have a hypoechoic mass at his abdominal ultrasonography. Abdominal computed tomography scan revealed a right well-defined suprarenal mass 7.7×7.3×5.8 cm in size. The diagnosis of EMH was confirmed with ultrasonographic-guided fine needle biopsy. Treatment options which include intensified regular blood transfusion and hydroxyurea have been started.

Extra medullary hematopoiesis associated to congenital dyserythropoietic anemia II in adult

The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis characterized by anemia with ineffective erythropoiesis and morphological abnormalities of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II or HEMPAS is the more frequent type. It is rare in adults. Extra medullary hematopoiesis is also a rare entity; it is a physiological response to chronic anemia observed in certain hemopathies like congenital dyserythropoietic anemia type II. We report the observation of a patient for who diagnosis of extra medullary hematopoiesis associated to congenital dyserythropoietic type II was made in adulthood.

Endoscopic ultrasound guided fine‐needle aspiration of a splenic hemangioma with extramedullary hematopoiesis

Extramedullary hematopoiesis (EMH) is the production of mature blood elements outside of the bone marrow and can occur as a compensatory result of a marrow replacing process or from marrow space occupying lesions such as tumor or marrow fibrosis. EMH can also be induced by factors elicited by neoplasms, such as vascular endothelial growth factor (VEGF). Usually, EMH is a diffuse process most commonly observed in lymph nodes, liver, and spleen. Rarely, EMH can form a mass lesion. Although the spleen is a common site for diffuse EMH, it is a rare location for a mass forming EMH. Hemangiomas are the most common benign tumors of the spleen. A case of a discrete, 8 cm lesion was noted incidentally on CT scan in a 59-year-old man with no significant past medical history. Endoscopic ultrasound guided fine-needle aspiration (EUS FNA) biopsy was performed and cytologic examination revealed trilinear hematopoiesis, with the most distinctive elements being megakaryocytes and erythroid precursors. A diagnosis of EMH was made. On resection, the mass was a hemangioma with EMH. EUS guided FNA is a useful tool for diagnosing splenic masses. Awareness of EMH, both as a mass forming lesion and a feature associated with benign and malignant vascular lesions is important, especially in patients with hematologic malignancies or marrow replacing processes.

Extramedullary hematopoiesis in unusual locations in hematologically compromised and noncompromised patients

Extramedullary hematopoiesis (EMH) occurs as a compensatory reaction to an underlying hematologic abnormality. EMH is most commonly seen in the liver and spleen but rarely has been reported in other locations, as well. On rare occasions, EMH may mimic a neoplasm in hematologically noncompromised patients. In this report, we present three cases of EMH in unusual locations, two in the presacral soft tissue and one in the synovial lining of the knee joint. The patients with presacral masses had no hematologic abnormality. In all patients with EMH, imaging plays an important role in both localization of the lesion and guidance for the biopsy; when imaging results are correlated with histological findings, the diagnosis of EMH can be confirmed.

Extramedullary hematopoiesis: A rare occurrence in the sinonasal tract

Extramedullary hematopoiesis (EMH) is a systemic reaction to inadequate hematopoiesis. We report two exceedingly rare cases of EMH involving the paranasal sinuses. The first patient, a 30-year-old man, presented with a maxillary sinus mass. The lesion was excised by endoscopic surgery: definitive histology identified foci of EMH within an inflammatory fibromyxoid pseudotumor. The second case occurred in a 29-year-old man affected by intermediate β-thalassemia. He was hospitalized with a diagnosis of sphenoid sinus mucocele secondary to an ethmoid lesion. The patient underwent endoscopic excision of the mass and drainage of the sphenoid mucocele. At definitive histology, a diagnosis of EMH was established. Herein, the presenting modalities, imaging profile, and treatment options of this rare EMH localization are reviewed.

Tc-99m nanocolloid scintigraphic imaging of intracranial meningeal extramedullary hematopoiesis in a patient with idiopathic myelofibrosis

Meningeal extramedullary hematopoiesis (EMH) is a rare finding in idiopathic myelofibrosis. Intracranial EMH is typically asymptomatic and sites are usually found by chance. Diagnosis of EMH is difficult, based on clinical circumstances and the use of different diagnostic imaging modalities, such as CT, MRI or radionuclide imaging. We present a case with intracranial medullary hematopoiesis due to idiopathic myelofibrosis diagnosed with Tc-99m nanocolloid scintigraphy. Cranium SPECT images that were performed with Tc-99m nanocolloid showed increased radiotracer uptake in the bilateral parietal, bilateral frontal and left occipital bones and especially in falx cerebri of sinus sagittalis superior. In Tc-99m MDP bone scintigraphy, increased osteoblastic activity in the left frontal and parietal bones, in shoulders, knee and ankle joints, and in both metatarsal bones were seen. After gadodiamid injection, Tl weighted MRI showed diffuse contrast increased in the meningeal areas surrounding the brain. A biopsy of the mass revealed extramedullary hematopoiesis composed of erythroblasts, mature and immature myeloid cells, and megakaryocytes. It was deduced that these described foci of EMH.

Asymptomatic spherocytosis presenting with spinal cord compression

Extramedullary hematopoiesis (EMH) is a compensatory mechanism occurring in patients with chronic anemia, which occurs most frequently with thalassemia. The authors report the case of a 57-year-old man, with no history of clinical or hematological disease, presenting with spinal cord compression. Magnetic resonance (MR) imaging demonstrated a homogeneous posterior epidural mass extending from T-3 to T-6. Following decompressive surgery, the patient's symptoms improved. Histological analysis showed features consistent with a diagnosis of EMH. Subsequent workup was remarkable for an asymptomatic spherocytosis without anemia. There was no family history of anemia. An EMH-related presentation of mild spherocytosis has been described in the literature, but its epidural location led to spinal cord compression. The MR imaging features were suggestive of EMH, but in the presence of spinal cord compression and in the absence of a history of chronic anemia, the authors did not believe that nonsurgical management would have been reasonable.