Ann Thorac Surg 2004;77:323– 4
CASE REPORT TASSIOPOULOS ET AL HEMOTHORAX AND EXTRAMEDULLARY ERYTHROPOIESIS
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Hemothorax Due to Extramedullary Erythropoietic Masses Stergios Tassiopoulos, MD, Kostas Konstantopoulos, MD, Yannis Rombos, MD, and Athanassios Aessopos, MD
We describe a 27-year-old male patient suffering from -thalassemia intermedia who presented with a nontraumatic spontaneous hemothorax due to extramedullary hemopoietic foci. In reviewing the literature, four similar reports were found. The details of this unusual entity are discussed. (Ann Thorac Surg 2004;77:323– 4) © 2004 by The Society of Thoracic Surgeons
I
n recent years enormous progress in understanding molecular pathology and pathophysiology of thalassemias has been reported. This permitted application of ore effective modalities, resulting in better prognosis and improved survival [1]. However, prolongation of survival increased the appearance of late sequelae of the disease and brought to light complications unknown as yet. Among them, some constitute medical emergencies, demanding immediate treatment. Extramedullary masses complicating thalassemias, apart from the known complications, may also bleed and produce acute manifestations.
A 29-year-old male suffering from -thalassemia intermedia (genotype IVS-1/IVS1-6), with hematocrit at 25% and hemoglobin at 8 g/dL, maintained a rather normal physical activity. He was splenectomized at 3 years old; cholocystectomy was also performed at 8 years old. He was lost to the regular follow-up, so he did not receive appropriate treatment. During the last 2 years his liver manifested progressive enlargement (liver span up to 17 cm) and several paravertebral extramedullary foci were noted, the largest of them, localized at T12 level, measuring some 15 cm. Without a regular chelation therapy, ferritin values ranged from 3000 to 4000 ng/mL. No abnormalities of cardiac, hepatic, or renal functions were detected. The patient was admitted as an emergency because of pleural pain and progressively deteriorating dyspnea. On clinical examination a pleural effusion was detected and radiologically confirmed (Fig. 1). A high resolution computed tomographic (CT) scan revealed some hyperdense areas within the pleural fluid, and a marked haziness of borders of the largest extramedullary mass. These findings suggested bleeding from this mass (Fig. 2). Pleural aspiration revealed 1000 mL of bloody fluid Accepted for publication April 29, 2003. Address reprint requests to Dr Stergios Tassiopoulos, 12 Kotieou str, Athens 11521, Greece; e-mail:
[email protected].
© 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
CASE REPORTS
First Department of Internal Medicine, University of Athens, “Laikon” Hospital, Athens, Greece
Fig 1. Chest roentgenogram depicting pleural effusion and extramedullary hemopoietic foci (arrows).
(hematocrit 10% vs 19% of venous blood). At the same time, the pleural effusion rapidly recurred, necessitating repeated aspirations. A total of 5 L of pleural fluid was removed in six sessions within 15 days. A progressive fall in pleural fluid hematocrit value (10% to 5%, 4%, and 2.7%) indicated a gradual cessation of bleeding that mitigated the need for surgical intervention. During this time the patient received a total of 8 units of packed red cells. After discharge, he was put on a transfusion protocol together with oral hydroxyurea (1g ⫻ 2 p.o.). One year later the patient is apparently well; no pleural adhesions being evident. The size of the extramedullary foci underwent a marked regression.
Comment Beta-thalassemia intermedia is an extremely heterogeneous disease, both genetically and clinically. A wide variety of genotypes (homozygotes, double heterozygotes, and simple heterozygotes) may be present. Patients usually maintain a relatively acceptable hemoglobin level (7–9 g/dL) and for this reason they are not regularly transfused [1]. However, these patients are under a constant hypoxia due to decreased blood oxygen load and defective hemoglobin unload in the periphery (inappropriate intraerythrocytic adaptation in anemia, elevated fetal hemoglobin percentage). The constant and long lasting hypoxia leads to enhanced rate of erythropoiesis and increased intestinal iron absorption. The former imply a marked bone marrow hyperplasia (skeletal deformities) and extramedullary foci of hemopoiesis, resulting in spleen and liver enlargement or even tumorlike masses in several sites, mainly paraverterably. These masses frequently remain asymtpomatic, but, in certain patients they generate many syndromes due to pressure of the surrounding structures (nerves, spinal cord, vertebrae). 0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)00754-9
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CASE REPORT PAPAGIANNOPOULOS ET AL EMPYEMAS FROM COLOPLEURAL FISTULAS
Ann Thorac Surg 2004;77:324 – 6
References
CASE REPORTS
1. Lukens JN. The thalassemias and related disorders. In Wintrobe’s clinical hematology. 10th edition. Baltimore: Williams and Wilkins, 1999:1405– 48. 2. Smith PR, Manjoney DL, Teitcher JB, Choi KN, Braverman AS. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Chest 1988;94:658 –60. 3. Kupferschmid JP, Shahian DM, Villanueva AG. Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest 1993;103:974 –5. 4. Bartlett RP, Greipp PR, Tefferi A, Cupps RE, Mullan BP, Trastek VF. Extramedullary hematopoiesis manifesting as a symptomatic pleural effusion. Mayo Clin Proc 1995;70:1161–4. 5. Chu KA, Lai RS, Lee CH, Lu JY, Chang HC, Chiang HT. Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia. Thorax 1999;54: 466 –8. Fig 2. Thoracic computed tomographic scan; hyperdense areas within the pleural fluid and haziness of borders of the largest extramedullary mass (arrow), indicative of intrapleural bleeding.
The extramedullary foci consist of hemopoietic cells and adipose elements. They are not circumscribed by a capsule. The mass is extremely vascular and contains little fibrous tissue. This structure renders the mass prone to hemorrage. Our patient represents a case of spontaneous massive hemothorax. Only four similar cases are reported in the literature due to -thalassemia [2], myelofibrosis [3], agnogenic myeloid metaplasia [4], and ␣-thalassemia [5], which were subjected to different treatment. In our patient, despite the rapid pleural fluid reproduction, there was some indication of self-cessation of bleeding (regression of pleural fluid hematocrit values in successive punctures), permitting a more conservative approach. Our report illustrates a rare complication of extramedullary erythropoiesis and, hopefully, will bring about a better understanding of the disease. Namely: (1) Spontaneous bleeding may be a complication of extramedullary erythropoiesis. (2) Most reports face extramedullary erythropoiesis as a benign complication; however, the possibility of such a complication necessitates a reconsideration of this view. (3) In such patients where the hematocrit values of pleural fluid progressively decrease, surgical intervention may not be necessary; 2 of 4 reports in the literature described surgical intervention, whereas the other two did not. (4) All thus far published cases of extramedullary foci bleeding deal with the pleural cavity; the reason is not yet clear, but it may be related to preferential localization or specific tissue structure(s), rendering them vulnerable to rupture due to thoracic respiratory movements. (5) Conservative treatment (transfusions and hydroxyurea) may suffice for the regression of extramedullary erythropoiesis. (6) Whether extramedullary foci represent a contraindication to anticoagulants is a matter of speculation. © 2004 by The Society of Thoracic Surgeons Published by Elsevier Inc
Empyema Resulting From a True Colopleural Fistula Complicating a Perforated Sigmoid Diverticulum Kostas Papagiannopoulos, MMED, Dimitrios Gialvalis, MD, Ibrahim Dodo, MRCS, and Mike J. Darby, MD Department of Cardiothoracic Surgery and Radiology, Leeds General Infirmary, Leeds, United Kingdom
Empyemas developing after traumatic rupture of intraabdominal organs have been previously reported. We report a case of a true nontraumatic colopleural fistula following surgery for spontaneous rupture of a sigmoid diverticulum. The diagnosis was suspected by the presence of an air-containing tract seen in a computerized tomogram of chest and abdomen and was established with a contrast study. The empyema cavity was initially drained, followed by a laparotomy and fistulectomy with primary large bowel anastomosis and loop ileostomy. Although rare, colopleural fistulas present a diagnostic challenge and delayed management can lead to increased morbidity. (Ann Thorac Surg 2004;77:324 – 6) © 2004 by The Society of Thoracic Surgeons
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mpyemas following inflammatory bowel diseases or intestinal trauma could indicate a fistulous connection of bowel with the pleural cavity. Careful evaluation of the pleural fluid, computerized tomograms of chest and abdomen, and contrast studies could provide early, prompt diagnosis leading to appropriate management. A 46-year-old male smoker, with no significant past medical history, presented in the Accident and Emergency Department with an acute abdomen. An emergency laparotomy and Hartmann’s procedure was under-
Accepted for publication April 8, 2003. Address reprint requests to Mr Papagiannopoulos, Leeds General Infirmary, Jubilee Building, Level D, Great George St, Leeds LS1 3EX, UK; e-mail:
[email protected].
0003-4975/04/$30.00 doi:10.1016/S0003-4975(03)01378-X
