Ann Thorac Surg 2004;77:323-324
© 2004 The Society of Thoracic Surgeons
Case report
Hemothorax due to extramedullary erythropoietic masses
Stergios Tassiopoulos, MDa*,
Kostas Konstantopoulos, MDa,
Yannis Rombos, MDa,
Athanassios Aessopos, MDa
a First Department of Internal Medicine, University of Athens, "Laikon" Hospital, Athens, Greece
Accepted for publication April 29, 2003.
* Address reprint requests to Dr Shiraishi, Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Kyoto, Japan 602-8566.
e-mail: isao{at}koto.kpu-m.ac.jp
 |
Abstract
|
|---|
We describe a 27-year-old male patient suffering from ß-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.
|
Introduction
|
|---|
In 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 ß-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).
View larger version (104K):
[in this window]
[in a new window]
|
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.
|
|
Pleural aspiration revealed 1000 mL of bloody fluid (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 x 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).
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 ß-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:
- Spontaneous bleeding may be a complication of extramedullary erythropoiesis.
- Most reports face extramedullary erythropoiesis as a benign complication; however, the possibility of such a complication necessitates a reconsideration of this view.
- 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.
- 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.
- Conservative treatment (transfusions and hydroxyurea) may suffice for the regression of extramedullary erythropoiesis.
- Whether extramedullary foci represent a contraindication to anticoagulants is a matter of speculation.
|
References
|
|---|
- Lukens J.N. The thalassemias and related disorders. Wintrobe's clinical hematology, 10th edition Baltimore: Williams and Wilkins, 1999:1405-1448.
- Smith P.R., Manjoney D.L., Teitcher J.B., Choi K.N., Braverman A.S. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with thalassemia intermedia. Chest 1988;94:658-660.[Abstract/Free Full Text]
- Kupferschmid J.P., Shahian D.M., Villanueva A.G. Massive hemothorax associated with intrathoracic extramedullary hematopoiesis involving the pleura. Chest 1993;103:974-975.[Abstract/Free Full Text]
- Bartlett R.P., Greipp P.R., Tefferi A., Cupps R.E., Mullan B.P., Trastek V.F. Extramedullary hematopoiesis manifesting as a symptomatic pleural effusion. Mayo Clin Proc 1995;70:1161-1164.[Medline]
- Chu K.A., Lai R.S., Lee C.H., Lu J.Y., Chang H.C., Chiang H.T. Intrathoracic extramedullary haematopoiesis complicated by massive haemothorax in alpha-thalassaemia. Thorax 1999;54:466-468.[Abstract/Free Full Text]
This article has been cited by other articles:
|
|
|
|
 
A. Aessopos, S. Tassiopoulos, D. Farmakis, I. Moyssakis, M. Kati, K. Polonifi, and M. Tsironi
Extramedullary Hematopoiesis-Related Pleural Effusion: The Case of {beta}-Thalassemia
Ann. Thorac. Surg.,
June 1, 2006;
81(6):
2037 - 2043.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
Introduction
