North American Journal of Medical Sciences

LETTER TO EDITOR
Year
: 2013  |  Volume : 5  |  Issue : 1  |  Page : 75--76

Hemolytic anemia in a female patient presenting for routine cholecystectomy


Saifullah Khalid1, Samreen Zaheer2, Sufian Zaheer3, Mohammed Sheeraz Alam4, Mohd Khalid1,  
1 Department of Radiodiagnosis, Jawaharlal Nehru Medical College and Hospital, AMU, Aligarh, UP, India
2 Department of Radiotherapy, Jawaharlal Nehru Medical College and Hospital, AMU, Aligarh, UP, India
3 Department of Pathology, Jawaharlal Nehru Medical College and Hospital, AMU, Aligarh, UP, India
4 Department of Medicine, Jawaharlal Nehru Medical College and Hospital, AMU, Aligarh, UP, India

Correspondence Address:
Saifullah Khalid
Department of Radiodiagnosis, Jawaharlal Nehru Medical College and Hospital, AMU, Aligarh, UP
India




How to cite this article:
Khalid S, Zaheer S, Zaheer S, Alam MS, Khalid M. Hemolytic anemia in a female patient presenting for routine cholecystectomy.North Am J Med Sci 2013;5:75-76


How to cite this URL:
Khalid S, Zaheer S, Zaheer S, Alam MS, Khalid M. Hemolytic anemia in a female patient presenting for routine cholecystectomy. North Am J Med Sci [serial online] 2013 [cited 2020 Jan 22 ];5:75-76
Available from: http://www.najms.org/text.asp?2013/5/1/75/106220


Full Text

Dear Editor,

Gall stones are of common occurrence and frequently cause acute abdominal pain and are managed by cholecystectomy. Cholecystectomy, whether open or closed, is among the most commonly performed surgeries worldwide. The cause of cholelithiasis is infrequently investigated properly. We report a case of a middle-aged female patient who presented for routine laparoscopic cholecystectomy and pre-operative chest X-ray which lead to the diagnosis of hemolytic anemia as the cause of gall stones with compensatory extramedullary hematopoiesis (EMH).

A middle-aged female patient presented to the surgery out-patient department for routine laparoscopic cholecystectomy. She recently had an episode of acute epigastric pain and was diagnosed as cholecystitis with cholelithiasis on ultrasound abdomen. During the pre-anesthetic work-up for cholecystectomy, she was referred to the radiology department for routine chest X-ray. Her routine investigations were all normal except for mild anemia (Hb −9.8 gm/dl). X-ray of the chest Postero-anterior and Lateral view showed well-defined mediastinal masses manifesting as increased retro-cardiac paravertebral soft-tissue density [Figure 1]a and as spine sign on Lateral view [Figure 1]b. Detailed history for any chest complaints was negative except for mild dyspnea and occasional cough. There was no family history of any hematological disorder. The patient was advised computed tomography (CT) of the thorax for evaluation of retro-cardiac opacity. Contrast-enhanced CT showed multiple, well-defined, lobulated, bilateral paravertebral masses with no evidence of calcification or fat density within masses and mild homogenous enhancement [Figure 2]a and b. There was no evidence of underlying bone expansion or destruction or extension into neural foramina. The lesions displaced adjacent lung field suggesting extra-parenchymal location of the lesions. Additional significant findings seen on CT in this patient were presence of gall stones, hepatomegaly, and spleenomegaly [Figure 2]c and d. Based on these radiological findings of bilateral posterior mediastinal masses; Lymphoma, Multiple Myeloma, Metastasis, EMH and Pott's spine were important differentials considered in this patient. Further hematological work-up of the patient revealed - Hemoglobin 9.8 gm/dl, hematocrit 35.6%, mean red cell volume 102 fl with increased reticulocyte count, i.e., 8%. The blood film contained about 28% spherocytes with markedly increased erythrocyte osmotic fragility on osmotic fragility lysis test, thereby suggesting the diagnosis of hereditary spherocytosis (HS). There was no evidence of any clinically palpable lymphadenopathy or retroperitoneal lymph nodes on ultrasound or CT. CT-guided fine-needle aspiration cytology of the paravertebral mass was performed which showed aggregates of erythroid and myeloid precursors with usual morphology along with megakaryocytes suggesting extramedullary hematopoiesis. Therefore, the diagnosis of HS with compensatory EMH was confirmed.{Figure 1}{Figure 2}

HS is the most common red cell membrane disorder with an autosomal dominant or, less commonly, an autosomal recessive pattern of inheritance. The peripheral blood smears show spherocytes with an increase in osmotic fragility. The pattern of expression of this disease is highly variable. It varies from clinically asymptomatic condition to a severe, life-threatening presentation. [1],[2]

The cause of EMH in HS, or for that matter in all hemolytic anemia, is a compensatory mechanism in response to destruction of red blood cells leading to the production of myeloid and erythroid elements at ectopic sites. Other conditions that predispose to EMH include myelofibrosis, polycythemia rubra vera, and replacement of bone marrow by carcinoma or lymphoma. EMH commonly occurs in spleen, liver, lymph nodes, and mediastinum and less commonly in skull, kidney, or retroperitoneum. [1],[2],[3],[4] Intrathoracic EMH is a relatively rare entity, commonly seen in the posterior mediastinum, and are frequently asymptomatic. They are frequently identified incidentally, as in our case. Rarely, they can produce symptoms of spinal cord compression if they extend to an epidural location. [5],[6] The investigation of choice for EMH is magnetic resonance imaging which shows an isointense mass with a high signal intensity rim on T1-weighted images and a homogenous hyper intensity on T2-weighted images. [3],[6]

Treatment of patients with EMH is considered only if complications occur. Treatment options are surgery, radiation therapy, blood transfusions, or their various combinations. [6] Relatively small doses of radiotherapy are quite effective as EMH masses are highly radiosensitive. [6],[7] Surgical removal of spleen is considered the treatment of choice for patients with EMH due to HS because it eliminates the main site of red cell destruction. [8]

This case highlights the importance of pre-operative chest X-ray in a patient having symptomatic gallstones and an un-investigated mild anemia which lead us to the diagnosis of HS with EMH. The routine pre-operative chest X-rays, however, still remains a debated issue and probably unjustified if we do it in all the cases. [9]

References

1Xiros N, Economopoulos T, Papageorgiou E, Mantzios G, Raptis S. Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis. Ann Hematol 2001;80:38-40.
2De Backer AI, Zachée P, Vanschoubroeck IJ, Mortelé KJ, Ros PR, Kockx MM. Extramedullary paraspinal hematopoiesis in hereditary spherocytosis. JBR-BTR 2002;85:206-8.
3Gabata T, Kadoya M, Mori A, Kobayashi S, Sanada J, Matsui O. MR imaging of focal splenic extramedullary hematopoiesis in polycythemia vera: Case report. Abdom Imaging 2000;25:514-6.
4Gogia P, Goel R, Nayar S. Extramedullary paraspinal hematopoiesis in hereditary spherocytosis. Ann Thorac Med 2008;3:64-6.
5Aydingöz U, Oto A, Cila A. Spinal cord compression due to epidural extramedullary haematopoiesis in thalassaemia: MRI. Neuroradiology 1997;39:870-2.
6Munn RK, Kramer CA, Arnold SM. Spinal cord compression due to extramedullary hematopoiesis in beta-thalassemia intermedia. Int J Radiat Oncol Biol Phys 1998;42:607-9.
7Malik M, Pillai LS, Gogia N, Puri T, Mahapatra M, Sharma DN, et al. Paraplegia due to extramedullary hematopoiesis in thalassemia treated successfully with radiation therapy. Haematologica 2007;92:e28-30.
8Reman O, Carre G, Belloy F, Galateau F, Cheze S, Macro M, et al. Extramedullary haematopoiesis in hereditary spherocytosis simulating mediastinal tumour. Eur J Haematol 1997;58:124-6.
9Mohammed TL, Kirsch J, Amorosa JK, Brown K, Chung JH, Dyer DS, et al. ACR appropriateness criteria® routine admission and preoperative chest radiography. Expert Panel on Thoracic Imaging. Reston (VA): American College of Radiology (ACR); 2011.