Radiological Features of Extramedullary Hematopoiesis in a Young Male With Beta-Thalassemia: A Case Report.

The formation of the blood elements and their maturation is called hematopoiesis. In adults, this typically takes place in the bone marrow of vertebrae, ribs, and long bones. In contrast, during fetal development, the primary sites of hematopoiesis are the spleen, liver, and the yolk sac. This process of hematopoiesis, when it occurs in sites other than the bone marrow, is called the extramedullary hematopoiesis (EMH). Extramedullary hematopoiesis usually happens in patients with blood disorders like sickle cell disease and thalassemia, where there is failure of hematopoiesis in the primary sites. Here, we present a young male with beta-thalassemia who presented with shortness of breath and palpitations for one month. This manuscript discusses the imaging findings of the EMH in our patient.

A Rare Presentation of Extramedullary Hematopoiesis as an Adrenal Mass: A Case Report.

Hematopoiesis is an enormous and complex process. When the primary site of hematopoiesis fails to meet the requirements of the body in conditions like hemoglobinopathies or myelofibrosis, various extramedullary sites take on the role of blood formation. Extramedullary hematopoiesis most commonly occurs in the liver, spleen, and lymph nodes and is rarely found in the thymus, heart, breast, adrenal glands, paravertebral regions, intraspinal tissue, and brain. Extramedullary hematopoiesis can mimic neoplasms in which symptoms are caused by the mass effect of the lesion. We report a rare case of a 41-year-old female patient with a fibrohematopoietic adrenal mass mimicking a neoplasm for which she underwent an adrenalectomy.

A Rare Case of Cord Compression From Extra-Medullary Hematopoiesis (EMH) in Beta Thalassemia.

Cord compression can arise from many different etiologies -- including trauma, degenerative changes, growths, neoplasms, or even abscesses. While some etiologies can cause symptoms such as weakness or motor deficits, others can simply present as pain. A rare cause of cord compression is extramedullary hematopoiesis (EMH), or the growth of blood cells outside the bone marrow. This rare, abnormal growth of cells can result in severe complications such as increased intracranial pressure and motor and sensory impairment. General clinicians should strive for early and prompt diagnosis of cord compression whenever possible, especially in patients who present with acute neurological deficits. We present a case of a 27-year-old female with beta thalassemia major (BTM) and transfusional hemosiderosis, who came in with progressive lower extremity weakness, numbness and urinary retention, and was diagnosed with acute cord compression from EMH.

Coexisting Extra-Medullary Manifestation of Chronic Myelomonocytic Leukemia and Follicular Lymphoma: What's Between Neoplastic Follicles Matters.

We illustrate a rare case of coexisting extramedullary manifestation of CMML and new onset follicular lymphoma within the same core-needle biopsy of a lymph node. We discuss the differences between extramedullary hematopoiesis and extramedullary manifestation of myeloid neoplasms. We also highlight the importance of generous tissue sampling and thorough examination of nodal tissue in the setting of an established myeloid neoplasm to avoid missing rare but possible nodal involvement.

[Re-irradiation of bone marrow compression on a focus of extra medullary hematopoiesis: Presentation of a case]. / Ré-irradiation de compression médullaire sur foyer d'hématopoïèse extra médullaire : présentation d'un cas.

We present the case of a 40-year-old patient with ß-Thalassemia major who underwent decompressive palliative radiotherapy on symptomatic intracanal extra medullary hematopoiesis on three occasions between 2016 and 2020. We delivered normo-fractionated radiotherapy at a dose of 10Gy in five fractions on vertebrae T4 to T10 and L3 to S2, 20Gy in ten fractions on vertebrae C7 to T11, and 12Gy in six fractions on vertebrae C7 to T9. The treatment was well tolerated with complete recovery of the symptomatology during the first two irradiations, and almost complete recovery after the 3rd irradiation.

Alleviation of acute radiation-induced bone marrow failure in mice with human fetal placental stromal cell therapy.

PURPOSE: Selected placental mesenchymal stromal cells isolated from the fetal mesenchymal placental tissues (f-hPSCs) were tested as cell therapy of lethal acute radiation syndrome (ARS) with bone marrow regeneration and induced extramedullary hematopoiesis. METHODS AND MATERIALS: f-hPSCs were isolated from the chorionic plate of human placentae and further expanded in regular culture conditions. 2 × 106 f-hPSCs were injected on days 1 and 4 to 8-Gy total body irradiated (TBI) C3H mice, both intramuscularly and subcutaneously. Pre-splenectomized TBI mice were used to test the involvement of extramedullary spleen hematopoiesis in the f-hPSC-induced hematopoiesis recovery in the TBI mice. Weight and survival of the mice were followed up within the morbid period of up to 23 days following irradiation. The role of hematopoietic progenitors in the recovery of treated mice was evaluated by flow cytometry, blood cell counts, and assay of possibly relevant growth factors. RESULTS AND CONCLUSIONS: The survival rate of all groups of TBI f-hPSC-treated mice at the end of the follow-up was dramatically elevated from < 10% in untreated to ~ 80%, with a parallel regain of body weight, bone marrow (BM) recovery, and elevated circulating progenitors of blood cell lineages. Blood erythropoietin levels were elevated in all f-hPSC-treated mice. Extramedullary splenic hematopoiesis was recorded in the f-hPSC-treated mice, though splenectomized mice still had similar survival rate. Our findings suggest that the indirect f-hPSC life-saving therapy of ARS may also be applied for treating other conditions with a failure of the hematopoietic system and severe pancytopenia.

Pulmonary extra-medullary hematopoiesis and pulmonary hypertension from underlying polycythemia vera: a case series.

Myeloproliferative neoplasia (MPN)-associated pulmonary hypertension (PH) is included in group five of the most recent clinical classification of PH.1 The MPNs are a heterogeneous group of disorders that includes disorders with primary expression of a myeloid phenotype and disorders characterized by expression of the Janus Kinase 2 (JAK2) mutation, p.V617F. The latter includes essential thrombocytosis, polycythemia vera, and idiopathic myelofibrosis.2 Pulmonary extra-medullary hematopoiesis (EMH) refers to the presence of hematopoietic precursor cells in the lung. It is a rare complication associated with myelofibrosis. Here we present a case series highlighting the clinical-pathological-radiological features of pulmonary EMH and PH from underlying polycythemia vera.

Adrenal extramedullary hematopoiesis associated with beta-thalassemia trait in an adult woman: a case report and review of literature.

INTRODUCTION: Adrenal myelolipoma with extramedullary hematopoiesis (EMH) is a rare condition. CASE PRESENTATION: We report a case of 48-year-old woman suffering from recurrent right hypochondrial pain for two years. Laboratory work-up revealed chronic microcytic iron deficiency anemia with beta thalassemia trait, right adrenal myelolipoma with histopathological evidence of hematopoietic cells (EMH). The patient underwent open resection of the adrenal gland and the post-operative course was uneventful. CONCLUSIONS: EMH could be presented with several clinical hematological disorders. Surgical management becomes inevitable in certain adrenal EMH cases especially in the presence of a large adrenal mass.

Renal Bleeding Due to Extramedullary Hematopoiesis in a Patient With Chronic Myelogenous Leukemia.

Chronic myelogenous leukemia (CML) is a myeloproliferative disorder that normally presents in middle-aged adults. Renal infiltration and extramedullary hematopoiesis in renal tissue has been rarely reported. This case report presents a patient with CML and renal insufficiency who developed gross hematuria. Efforts at controlling the hematuria led to a cascade of events propelled by the underlying disorder that ultimately led to a radical nephrectomy, multiorgan failure, and prolonged hospitalization. We suggest that management of gross hematuria in clinically stable patients with CML, suspected of having extramedullary hematopoiesis, should prioritize treatment of the myeloproliferative disorder over efforts to control bleeding.

Case report of the extramedullary hematopoiesis presented as a hypervascular intracranial mass.

Thalassemia is a hematologic disorder that causes ineffective hematopoiesis and is related to severe anemia, iron overload, extramedullary hematopoiesis, and hepatomegaly. Hepatomegaly is related to significant extramedullary hematopoiesis. The other sites that are involved in extramedullary hematopoiesis are spleen, lymph nodes, paraspinal regions, kidney, pleura, and intestine, but intracranial involvement is a rare presentation. We discuss about a case with intracranial medullary hematopoiesis in a thalassemic patient.