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A lump on the head
Case Report
A lump on the head Mehran Karimi, Nader Cohan, Mohamad Hadi Bagheri, Mehrzad Lotfi, Shapour Omidvari, Bita Geramizadeh Lancet 2008; 372: 1436 Haematology Research Centre (Prof M Karimi MD, N Cohan MSc), Department of Radiology, Nemazee Hospital (M H Bagheri MD, M Lotfi MD), Department of Radiotherapy, Nemazee Hospital (S H Omidvari MD), and Organ Transplant Research Centre and Department of Pathology (Prof B Geramizadeh MD), Shiraz University of Medical Science, Shiraz, Iran Correspondence to: Prof Mehran Karimi, Paediatric Haematology-Oncology, Haematology Research Centre, Shiraz University of Medical Science, Shiraz, Iran [email protected]
A
In December, 2007, a 23-year-old man was referred to our hospital with a left temporal mass (figure). The mass had first appeared 8 months before, and had steadily increased in size. When 7 years old, the patient had been diagnosed with β-thalassaemia, for which he had received blood transfusions: initially every 2–3 months, then every 4–8 weeks. Before transfusions, his blood haemoglobin concentration was 80–85 g/L; the ferritin concentration was around 5500 pmol/L. He had adhered only sporadically to treatment with desferrioxamine, because he found the infusions painful. When he was 7 years old, his enlarged spleen had been removed; when he was 22, his gallbladder had been removed, because of gallstones. The mass was not tender. CT of the head showed the mass to consist of soft tissue, and depicted widening of the diploic space. MRI with contrast showed a softtissue mass extending into the periphery of the orbit. B
DD
We took a sample of the mass by fine-needle aspiration; cytological analysis showed megakaryocytes, nucleated red blood cells, and precursors of myeloid white-blood cells. We diagnosed extramedullary haematopoiesis. We gave the patient low-dose radiotherapy (1600 centigrays in 8 fractions: 200 centigrays per fraction, 5 fractions per week), and prescribed hydroxyurea, at a dose of 500 mg per day. By the end of the treatment course, the lump had disappeared. When last seen, in March, 2008, the patient was well, and continued to receive blood transfusions for thalassaemia. Extramedullary haematopoiesis occurs, as a compensatory phenomenon, when bone-marrow function is impaired, in diseases including thalassaemia major or intermedia, sickle cell disease, myelofibrosis, and polycythaemia;1 cancer, such as leukaemia or lymphoma, should be considered as an important differential diagnosis.1,2 Extramedullary haematopoiesis is most commonly found in the liver, spleen, and lymph nodes, but has also been reported in the pleura, pericardium, intracranial cavity, adrenal glands, breast, presacral space, intrathoracic cavity, kidney, tear gland, and spinal cord.1,3–5 It is more common in patients who do not receive blood transfusions, than in those who do. We aim to keep this patient’s haemoglobin concentration above 100 g/L, to reduce the likelihood that extramedullary haematopoiesis will recur. Considerable debate continues about the best way to manage the disorder: management can vary according to the site of the haematopoietic tissue. Acknowledgments We thank Shiraz University of Medical Science, for supporting our work. We also thank the patient.
C
Figure: Extramedullary haematopoeisis (A) The patient at presentation. (B) MRI scan, showing the tumour (arrow). (C) Cytological smear (Wright’s stain; magnification×100). A white-blood-cell precursor, megakaryocyte, and nucleated red blood cell are identified by a filled arrow, open arrow, and arrowhead, respectively. (D) The patient after treatment.
1436
References 1 Turgut B, Pamuk GE, Vural O, et al. An interesting presentation of intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Clin Lab Haematol 2003; 25: 409–12. 2 Gibbins J, Pankhurst T, Murray J, et al. Extramedullary haematopoiesis in the kidney: a case report and review of literature. Clin Lab Haematol 2005; 27: 391–94. 3 Shinder R, Mirani N, Wu HV, Langer PD. Extramedullary hematopoiesis in the lacrimal gland. Ophthal Plast Reconstr Surg 2008; 24: 48–50. 4 Chiam QL, Lau KK. Extramedullary haematopoiesis in thalassaemia major causing spinal cord compression. Australas Radiol 2007; 51: 168–71. 5 Jabr FI, Aoun E, Yassine H, Azar C, Taher A. Beta-thalassemia intermedia and Hodgkin lymphoma. Am J Hematol 2006; 81: 151.
www.thelancet.com Vol 372 October 18, 2008
A lump on the head Mehran Karimi, Nader Cohan, Mohamad Hadi Bagheri, Mehrzad Lotfi, Shapour Omidvari, Bita Geramizadeh Lancet 2008; 372: 1436 Haematology Research Centre (Prof M Karimi MD, N Cohan MSc), Department of Radiology, Nemazee Hospital (M H Bagheri MD, M Lotfi MD), Department of Radiotherapy, Nemazee Hospital (S H Omidvari MD), and Organ Transplant Research Centre and Department of Pathology (Prof B Geramizadeh MD), Shiraz University of Medical Science, Shiraz, Iran Correspondence to: Prof Mehran Karimi, Paediatric Haematology-Oncology, Haematology Research Centre, Shiraz University of Medical Science, Shiraz, Iran [email protected]
A
In December, 2007, a 23-year-old man was referred to our hospital with a left temporal mass (figure). The mass had first appeared 8 months before, and had steadily increased in size. When 7 years old, the patient had been diagnosed with β-thalassaemia, for which he had received blood transfusions: initially every 2–3 months, then every 4–8 weeks. Before transfusions, his blood haemoglobin concentration was 80–85 g/L; the ferritin concentration was around 5500 pmol/L. He had adhered only sporadically to treatment with desferrioxamine, because he found the infusions painful. When he was 7 years old, his enlarged spleen had been removed; when he was 22, his gallbladder had been removed, because of gallstones. The mass was not tender. CT of the head showed the mass to consist of soft tissue, and depicted widening of the diploic space. MRI with contrast showed a softtissue mass extending into the periphery of the orbit. B
DD
We took a sample of the mass by fine-needle aspiration; cytological analysis showed megakaryocytes, nucleated red blood cells, and precursors of myeloid white-blood cells. We diagnosed extramedullary haematopoiesis. We gave the patient low-dose radiotherapy (1600 centigrays in 8 fractions: 200 centigrays per fraction, 5 fractions per week), and prescribed hydroxyurea, at a dose of 500 mg per day. By the end of the treatment course, the lump had disappeared. When last seen, in March, 2008, the patient was well, and continued to receive blood transfusions for thalassaemia. Extramedullary haematopoiesis occurs, as a compensatory phenomenon, when bone-marrow function is impaired, in diseases including thalassaemia major or intermedia, sickle cell disease, myelofibrosis, and polycythaemia;1 cancer, such as leukaemia or lymphoma, should be considered as an important differential diagnosis.1,2 Extramedullary haematopoiesis is most commonly found in the liver, spleen, and lymph nodes, but has also been reported in the pleura, pericardium, intracranial cavity, adrenal glands, breast, presacral space, intrathoracic cavity, kidney, tear gland, and spinal cord.1,3–5 It is more common in patients who do not receive blood transfusions, than in those who do. We aim to keep this patient’s haemoglobin concentration above 100 g/L, to reduce the likelihood that extramedullary haematopoiesis will recur. Considerable debate continues about the best way to manage the disorder: management can vary according to the site of the haematopoietic tissue. Acknowledgments We thank Shiraz University of Medical Science, for supporting our work. We also thank the patient.
C
Figure: Extramedullary haematopoeisis (A) The patient at presentation. (B) MRI scan, showing the tumour (arrow). (C) Cytological smear (Wright’s stain; magnification×100). A white-blood-cell precursor, megakaryocyte, and nucleated red blood cell are identified by a filled arrow, open arrow, and arrowhead, respectively. (D) The patient after treatment.
1436
References 1 Turgut B, Pamuk GE, Vural O, et al. An interesting presentation of intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Clin Lab Haematol 2003; 25: 409–12. 2 Gibbins J, Pankhurst T, Murray J, et al. Extramedullary haematopoiesis in the kidney: a case report and review of literature. Clin Lab Haematol 2005; 27: 391–94. 3 Shinder R, Mirani N, Wu HV, Langer PD. Extramedullary hematopoiesis in the lacrimal gland. Ophthal Plast Reconstr Surg 2008; 24: 48–50. 4 Chiam QL, Lau KK. Extramedullary haematopoiesis in thalassaemia major causing spinal cord compression. Australas Radiol 2007; 51: 168–71. 5 Jabr FI, Aoun E, Yassine H, Azar C, Taher A. Beta-thalassemia intermedia and Hodgkin lymphoma. Am J Hematol 2006; 81: 151.
www.thelancet.com Vol 372 October 18, 2008