Case Of Extramedullary Hematopoiesis Research Articles

Imaging features in extramedullary hematopoiesis - A case series

Extramedullary hematopoiesis (EMH) is a rare compensatory process associated with many hematologic disorders and bone marrow dysfunction. Through this article, we aim to present various imaging features of EMH and its complications including spinal cord compression. Three cases of EMH in hemoglobinopathies (thalassemia and sickle cell anemia) have been described. Cases 1 and 2 of our series include paraspinal masses causing cord compression. Case 1 of thalassemia intermedia presented with various skeletal deformities in long bones, skull, and facial bones (Chipmunk facies). Case 2 was diagnosed with extrapleural lung mass with other paraspinal lesions. Case 3 presented with multiple enlarged retroperitoneal lymph nodes due to EMH. Common and uncommon imaging features of EMH have been described. Lymph node involvement was difficult to diagnose as it mimicked tubercular and neoplastic etiologies; hence, histopathological diagnosis was mandatory.

Extramedullary hematopoiesis involving the thyroid: Fine-needle aspiration features and literature review

The thyroid has rarely been documented as a site of extramedullary hematopoiesis (EMH). We report the largest series to date, with nine cases of EMH, and compare our findings with previous reports of thyroid EMH. Thyroid nodule fine-needle aspirations (FNAs) were collected over a 4-year period. Thin layer preparations were examined and correlated with clinical features and ultrasound characteristics. A comprehensive review of the English literature was done, and the results were compared with the current series. During the study period, 172,939 thyroid FNAs were examined. Nine samples (0.005%) contained trilineage bone marrow elements. Nodule calcifications were present in 7 patients. None of the patients had a history of a blood disorder, nor was there any evidence of a thyroid malignancy. Fifteen reports of 18 patients with thyroid EMH were identified in the English literature. Nodule calcifications were reported in 10 patients. Thyroid EMH was associated with primary myelofibrosis in 4 patients, and with chronic anemia in 1; calcifications were absent in 3 patients, and were not specified in the remaining 2. None had evidence of a thyroid malignancy. Thyroidal EMH is an extremely uncommon finding. Clinical and sonographic features are nonspecific. Thyroid EMH is usually an incidental finding, most likely related to mature osseous metaplasia. An occasional association with hematologic disease has been reported, however. To date, there does not appear to be any association with thyroid malignancy and surgery is typically not indicated. Therefore, recognition at the time of FNA is essential.

Hématopoïèse extra-médullaire symptomatique au cours des β-thalassémies : étude rétrospective monocentrique

IntroductionSymptomatic extramedullary hematopoiesis (EH) is a rare but potentially severe phenomenon which occurs in β-thalassemia. There are no treatment guidelines. MethodsRetrospective single centre study including the cases of symptomatic EH encountered between 1997 and 2014 in a unit specialised in red blood cell genetic disorders. Description of clinical, biological and radiological characteristics of the patients, treatments received, and outcomes. ResultsAmong 182 β-thalassemia patients followed during the study period, 7 cases of symptomatic EH were diagnosed. They were 5 men and 2 women, and their mean age was 37 years. Four patients were splenectomised, two patients were regularly transfused, and four patients had already received erythropoietin. EH was localised in intravertebral areas and responsible for dorsal spinal cord compression in 5 patients, in paravertebral dorsal area in 1 patient, and in presacral area in 1 patient. The mean hemoglobin level at diagnosis was 7.9 g/dL. Treatment administered included: red cell transfusion in 6 cases, associated with hydroxyurea in 5 cases and/or radiotherapy in 3 patients. One patient was treated with surgery and HU. After a median follow-up of 41 months, clinical recovery was complete in 2 patients and partial in 5 patients. ConclusionEH must be suspected in β-thalassemia in patients presenting clinical signs of organ compression, and a typical radiological aspect. The functional prognosis depends on the rapidity of treatment, which includes red blood cell transfusion, hydroxyurea, radiotherapy, and rarely surgery. Long-term outcome is uncertain.

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.

Extramedullary haematopoiesis in spleen of Wistar rat: A case report

A case of extramedullary haematopoiesis in spleen has been reported in Wistar rat. Grossly, spleen was severely enlarged with rounded borders. Histomorphology exhibited marked erythropoiesis with megakaryocytosis.

Extramedullary hematopoiesis in renal allograft

Extramedullary hematopoiesis (EMH), defined as the presence of hematopoietic elements outside of the medullary cavity of bone, has been reported in patients with various hematopoietic neoplasms including myelofibrosis. EMH commonly occurs in the liver and spleen (resulting in hepatosplenomegaly) and uncommonly involves the kidney. EMH involving the allograft kidney has not been reported in English literature. Herein, we report the first case of EMH in allograft kidney in a patient with myelofibrosis. The clinical and pathological findings are described. Through comparison of the medullary neoplastic infiltrate with the renal allograft infiltrate, we postulate the neoplastic nature of the infiltrate in the allograft kidney.

Leiomyoma With Extramedullary Hematopoiesis and Intravascular Thrombosis

We report a case of extramedullary hematopoiesis (EMH) in uterine leiomyoma associated with numerous intravascular thrombi. A 29-year-old female, gravida 0, para 0, presented with heavy vaginal bleeding. Her hematocrit was 22%. She had a documented history of uterine leiomyomata and menorrhagia for a year. Transvaginal ultrasound confirmed the presence of a …

A blood-making lung mass

Dear Editor, An 81-year-old woman presented to us with right arm weakness and slurred speech. She had a history of rheumatoid arthritis, chronic renal insufficiency, hypertension, and a monoclonal gammopathy of undetermined significance. Her vital signs and physical examination were unremarkable. A complete blood count with differential showed hemoglobin 10.7 g/dL, platelets 493,000/μL, 68 % neutrophils, 12 % lymphocytes, 19 % monocytes, 1 % eosinophils, 0 % basophils, and 0.3 % immature granulocytes. Peripheral blood smear was notable for excessive platelets, frequent platelet clumping, giant platelets, normochromic normocytic red blood cells, and normal-appearing white blood cells. A leukoerythroblastic picture was not present. The majority of her platelet counts had been greater than 500,000/μL for the past 6 months. A comprehensive metabolic panel showed creatinine 1.7 mg/dL. A CT scan of head showed no evidence of acute infarct or hemorrhage. A carotid Doppler showed no evidence of hemodynamically significant stenosis. The patient’s right arm weakness and slurred speech resolved in 3 h. This episode was diagnosed with transient ischemic attack (TIA). She was given Plavix and aspirin. Subsequently, a chest X-ray incidentally revealed a mass in her right lung (Fig. 1a). A CT scan of chest showed an 8.4× 5.4×7.3 cm heterogeneous mass in her right hemithorax (Fig. 1b). A CT scan-guided percutaneous biopsy of the lung mass showed cellular hematopoietic elements within the trilineage maturation (Fig. 1c), indicating that the mass was due to extramedullary hematopoiesis (EMH). The bone marrow aspiration and biopsy were obtained without difficulty. Bone marrow specimens revealed maturing trilineage hematopoiesis with a marked myeloid predominance, megakaryocytic atypia (Fig. 1d), and minimal reticulin fibrosis (Fig. 1e). Her JAK-2 mutation analysis was positive, and the presence of JAK-2 mutation ruled out secondary myelofibrosis caused by toxin, infection, and other hematological malignancies. Bone marrow biopsy findings and presence of JAK-2 mutation were consistent with the diagnosis of prefibrotic phase of idiopathic myelofibrosis. The patient was started on hydroxyurea, continued on Plavix, and discharged after 4 days; at the last follow-up in August, 2012, she had no further similar TIA, platelet counts were normal, and she was well. Other than in the liver and spleen, EMH is exceptionally rare. To date, there have been a few reported cases of pulmonary EMH. Rumi et al. presented a case of dyspnea secondary to pulmonary EMH [1]. Hsu et al. reported a case of EMH mimicking metastatic lung carcinoma [2]. In another case report, Pandit et al. described a pulmonary EMH induced by chronic allergen challenge [3]. Here, we report a case of a patient with a pulmonary EMH in the setting of prefibrotic phase of idiopathic myelofibrosis. The presence of JAK-2 mutation in our patient ruled out secondary myelofibrosis caused by toxin, infection, and other hematological malignancies. Decision regarding management of pulmonary EMH depends on symptoms, coexisting conditions, and underlying causes. There are no set guidelines on the treatment of pulmonary EMH. Cytoreduction with hydroxyurea, steroids, allogenic stem cell transplantation, and radiation therapy have been documented in some case reports with varying success [4]. Our patient received cytoreductive treatment (hydroxyurea). Her platelets counts came down to normal, and her clinical course was stable. J. Wang (*) Department of Internal Medicine, St. Luke’s Hospital, 232 South Woods Mill Road, Chesterfield, MO 63017, USA e-mail: Jianbo310@gmail.com

Lymph Node Extramedullary Hematopoiesis in Breast Cancer Patients Receiving Neoadjuvant Therapy

Extramedullary hematopoiesis (EMH) develops as a compensatory mechanism associated with hematologic processes but it may occur in association with chemotherapy. Three cases of EMH arising in axillary lymph nodes following neoadjuvant therapy for breast carcinoma are reported herein. Three women ranging in age from 41 to 47 years presented with unilateral breast masses measuring 0.6 to 4.0 cm in greatest dimension and were diagnosed with infiltrating ductal carcinoma, grade III by core needle biopsies. Two of the tumors were triple negative and one was estrogen receptor positive. All patients subsequently received neoadjuvant therapy followed by lumpectomies. No residual carcinoma was identified in postchemotherapy breast resection specimens. One patient underwent a sentinel lymph node procedure, the second patient an axillary lymph node dissection, and the third patient had a core biopsy of an enlarged axillary lymph node. The patient that underwent axillary lymph node dissection had metastatic carcinoma in one of her lymph nodes. Foci of EMH consisting of myeloid, erythroid, and megakaryocytic precursors were present within the nodal parenchyma and/or subcapsular sinuses of axillary lymph nodes of all three cases. Megakaryocytes were immunoreactive with factor VIII, erythroid elements with Glycophorin and myeloid precursors with myeloperoxidase. With increasing use of neoadjuvant therapy for breast carcinoma, EMH within lymph nodes is more likely to be encountered. Hematopoietic precursors present in lymph nodes may potentially be misdiagnosed as metastatic tumor cells, particularly as lobular carcinoma or metaplastic carcinoma. Therefore, caution should be exercised when evaluating axillary lymph nodes in the clinical setting of neoadjuvant therapy for breast carcinoma.

A Typical Extramedullary Haematopoiesis in a JAK2 Mutated Primary Myelofibrosis Patient after a Minor Head Injury

Intracranial extramedullary haematopoiesis (EMH) is very rare. We report the case of EMH with intracranial involvement which developed after trauma in a 73-year-old lady affected by JAK2 mutated myelofibrosis (MF). She was diagnosed with MF in 2005 and treated with low-dose-steroids (prednisolone and oxymethalone) for about 5 years with anexcellent response on full blood count and splenomegaly. In June 2011, she fell from the stairs and sustained minor trauma to the forehead. Six weeks later, the patient had a gradually enlarging and moderately painful lump in the centre of her forehead. A contrast-enhanced CT scan of the head was performed, showing a small fracture in the frontal bone infiltrated and surrounded by a soft tissue mass extending intracranially and invading the skin extracranially (Figures 1 and 2). A metastatic deposit from an occult tumour was suspected. In August 2011, we performed a CT-guided trucut biopsy of the forehead mass. The histology report confirmed extramedullary haematopoiesis according to the patchy expression of myeloid (CD45, CD43, myeloperoxidase) and megakaryocytic markers (CD68, CD42b). We concluded that the trauma caused a small fracture which triggered the bone marrow tissue reaction resulting in production of extramedullary haematopoiesis in the site of the trauma. The patient underwent local radiotherapy resulting in complete resolution of the lesion.

Extramedullary Hematopoiesis in the Uterine Cervix Associated with Tissue Repair

Extramedullary hematopoiesis (EMH) is the presence of hematopoietic precursors outside the bone marrow. This condition is usually associated with hematologic disorders. Although EMH can be found in almost every site in the body, female genital tract involvement is rare. The authors report EMH in the uterine cervix from a 64-year-old patient following cervical biopsy due to abnormal cervical cytology. Neither neoplasm nor hematologic disorder was detected before the diagnosis and after 1 year of follow up. To the best of our knowledge, this is the first reported case of EMH involving the uterine cervix which showed an association with tissue repair.

Acute Submandibular Tumour in Myelodysplastic Syndrome: A Rare Report

Myelodysplastic syndromes (MDS) are hematological diseases with a wide clinical spectrum of manifestations. We present a 66 year old patient who was referred to our department with an acute submandibular growth that was first noticed 2 weeks ago. It hasn’t responded to standard treatment. Multiple studies were performed, and they revealed in filtrate of mielodisplastic cells, refractory anemia with excess blasts in transformation (RAEB-T) subtype according to the FAB classification. Combined chemotherapy couldn’t stop the disease’s progression and the patient died from a pulmonary infection 3 months after the onset of symptoms. Leukemic infiltration of the submandibular gland in mielodisplastic syndrome hasn’t been previously described in literature, so this seems to be the first reported case of extra medullary hematopoiesis resulting in an acute submandibular tumor in a patient with REAB-T.

Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: A case of extramedullary hematopoiesis

Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease.

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 presented as solitary renal mass: A case report with review of literature

In this article, we described a case of extramedullary hematopoiesis (EMH) in a 12-year-old boy with the clinical and hematological features of hemolytic anemia of unknown cause. The patient presented with a solitary well circumscribed mass in right kidney. Ultrasound guided fine-needle aspiration cytology showed myelocytes, metamyelocytes, megakaryocytes, and immature erythroid cells. A cytological diagnosis of EMH was made.

Extramedullary hematopoiesis within the clivus: an unusual cause of lower cranial nerve palsy

We report one year follow up of a case of extramedullary hematopoiesis within the clivus. The imaging findings, brief clinical course, and endoscopic transphenoidal approach are described. A 29-year-old female with thalassemia developed worsening cranial nerve signs. After imaging studies discovered a large clival mass, she underwent endoscopic transphenoidal biopsy of the lesion. Neural compression from exuberant erythrogenesis within tissue normally quiescent of red blood cell production was found to be the etiology of her neural deficit. Treatment for this condition is generally non-operative unless significant neural compression is present. Radiotherapy and anti-neoplastic agents have been used with success.

Fine-needle aspiration of extranodal and extramedullary hematopoietic malignancies

There is relatively little information concerning the use of fine-needle aspiration (FNA) to diagnose extranodal and extramedullary hematopoietic malignancies. Seventy-one such cases diagnosed by FNA form the basis of this study. Seventy-one cases of FNAs performed between 1988 and 1998 on extranodal and extramedullary hematopoietic malignancies were reviewed in order to evaluate the usefulness of this technique in diagnosing these entities as well as to assess patterns of relapse. There were 45 male and 26 female patients ranging in age from 29-86 years (mean, 68 years). Sixty-six patients had a previous history of a hematopoietic malignancy. Aspirates from 65 of these patients were consistent with the patient's known primary. One aspirate of a paravertebral mass from a multiple myeloma patient showed extramedullary hematopoiesis. The remaining five aspirates were cases of multiple myeloma that first presented as soft tissue masses. The most common malignancies were lymphoma: 52 cases (73%), 48 large cell lymphomas, four mixed small and large cell lymphoma; followed by multiple myeloma: 12 cases (17%); leukemia: four cases (5.4%); Hodgkin disease: two cases (2.8%); and one case of extramedullary hematopoiesis. The aspirate sites were soft tissue: 23 cases (32%); bone: 17 cases (24%); kidney: 14 cases (20%); liver: 11 cases (15%); lung: three cases (4%); adrenal: two cases (3%); and eye: one case. The interval between primary diagnosis and FNA was 1-36 months (mean, 13 months). In conclusion, 98% of the aspirates were neoplastic in patients with a known history of hematopoietic malignancies. The most common site of involvement was soft tissue in 23 (32%) cases. In five patients with multiple myeloma, the FNA diagnosis prompted a work-up to find the primary site of involvement. FNA is a useful technique in assessing extranodal and extramedullary hematopoietic malignancies.

MR imaging of adrenal masses: value of chemical-shift imaging for distinguishing adenomas from other tumors.

CT and conventional MR imaging are helpful in characterizing adrenal tumors, but a specific diagnosis is not achieved for a substantial number of lesions. Chemical-shift imaging relies on the different resonance frequencies of protons in water and triglyceride molecules and therefore may permit a more specific diagnosis of adrenal adenomas, which are known to contain abundant lipid. The purpose of this study was to evaluate the usefulness of chemical-shift MR imaging in the differentiation of adrenal adenomas from other adrenal masses. Forty-one adrenal masses (17 nonhyperfunctioning adenomas, two aldosteronomas, six pheochromocytomas, one ganglioneuroma, five adrenal carcinomas, one lymphoma, seven metastases, one case of extramedullary hematopoiesis, and one leiomyosarcoma) suspected clinically or identified by sonography or CT in 38 patients were prospectively evaluated with MR imaging. Pathologic proof of diagnosis was obtained for 28 lesions, and stability on imaging follow-up (mean, 19 months) was accepted as proof of diagnosis of benign adenoma for 13 lesions. In-phase T1-weighted spin-echo sequences (500/20 [TR/TE]) and opposed-phase gradient-echo sequences (142/6.3, flip angle = 90 degrees) of the adrenal regions were applied. Quantitative analysis of signal intensity loss in the adrenal lesions relative to reference tissues (liver, muscle, and spleen) on in-phase and opposed-phase sequences was done to differentiate adenomas from nonadenomas. Region-of-interest signal intensity measurements were obtained in a standard fashion by selection of the largest possible representative sample. Using liver as the reference standard, we found that mean signal intensity ratios were 0.47 (range, 0.23-0.97) for adrenal adenomas and 0.88 (range, 0.65-1.32) for nonadenomas; signal intensity ratios for two adenomas overlapped those of the nonadenomas. Using muscle as the reference standard, we found that mean signal intensity ratios were 0.44 (range, 0.22-0.66) for adrenal adenomas and 0.85 (range, 0.59-1.39) for nonadenomas; signal intensity ratios for two adenomas overlapped those of the nonadenomas. Using spleen as the reference standard, we found that mean signal intensity ratios were 0.45 (range, 0.27-0.73) for adrenal adenomas and 0.97 (range, 0.8-1.18) for nonadenomas, with no overlap. The mean signal intensity ratios were significantly different between adenomas and nonadenomas for all three reference tissues (p < .001). Our results show that chemical-shift MR imaging is an important new technique that enables the differentiation of adrenal adenomas from other adrenal masses, reducing the need for biopsy and prolonged imaging follow-up in patients with adrenal tumors.

Massive mediastinal extramedullary hematopoiesis in hereditary spherocytosis: A case report

A case of extramedullary hematopoiesis (EMH) with a massive mediastinal mass in a 72-year-old woman with hereditary spherocytosis is reported. Several cases of EMH have been described in nonsplenectomized adults with hereditary spherocytosis, and it is thought to be a consequence of long-term stimulation of erythropoiesis. The exact mechanism remains controversial. EMH should be considered in the differential diagnosis of an asymptomatic paravertebral mass.

Spinal Cord Compression due to Extramedullary Hematopoiesis

MYELOFIBROSIS is not uncommon, but compression of the spinal cord due to extramedullary hematopoiesis in this condition is rare. Of the 11 cases of extramedullary hematopoiesis causing spinal cord compression in the spinal epidural space that have been reported in the literature, six were associated with myelofibrosis and five with thalassemia major. 1-5 We report the 12th case, which involved the successful treatment of a woman with longstanding myelofibrosis, who underwent surgical decompression followed by radiation treatment for this condition. Report of a Case A 74-year-old woman was diagnosed as having myelofibrosis by the results of a bone marrow biopsy in January 1981. Initial treatment with oxymetholone and folic acid was continued until April 1983, at which time prednisone was added because of persistent hemoglobin values of about 5 g/dL. On repeated office visits thereafter, the hemoglobin values were about 10 g/dL. There was no objective evidence of blood loss.