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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 1  |  Issue : 2  |  Page : 159-162

Extramedullary hematopoiesis presenting as a solitary renal mass and mimicking a malignant tumor: A rare case report


1 Department of Pathology, Sheri-kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India
2 Department of Urology, Sheri-kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India

Date of Web Publication13-Dec-2013

Correspondence Address:
Basharat Mubeen
Department of Pathology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir - 190 011
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.123035

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  Abstract 

Extramedullary hematopoiesis (EMH) is the development of hematopoietic tissue outside the bone marrow and it most often occurs in the liver and spleen. Renal EMH is quite rare, and there are very few case reports concerning the kidney. We describe a case diagnosed with congenital dyserythropoetic anemia presenting with a solitary renal mass with splenomegaly. CECT showed a heterogeneously enhancing mass lesion suggestive of renal neoplasm. Microscopic examination revealed features of extramedullary hematopoiesis. We intend to present this case because of the rarity of EMH in kidney and to emphasize that its possibility should be kept in mind in any case of solitary renal mass, especially in those patients suffering from chronic hematological disorders.

Keywords: Congenital dyserythropoetic anemia, extramedullary hematopoiesis, kidney


How to cite this article:
Mubeen B, Lone IM, Hameed A, Charak A, Feroz I. Extramedullary hematopoiesis presenting as a solitary renal mass and mimicking a malignant tumor: A rare case report. Arch Med Health Sci 2013;1:159-62

How to cite this URL:
Mubeen B, Lone IM, Hameed A, Charak A, Feroz I. Extramedullary hematopoiesis presenting as a solitary renal mass and mimicking a malignant tumor: A rare case report. Arch Med Health Sci [serial online] 2013 [cited 2019 Dec 14];1:159-62. Available from: http://www.amhsjournal.org/text.asp?2013/1/2/159/123035


  Introduction Top


Renal masses of uncertain etiology may be discovered incidentally, and in some cases, the diagnosis is difficult to establish based only on imaging examination. Extramedullary hematopoiesis (EMH) refers to the development of hematopoietic tissue outside the bone marrow and normally occurs in the reticulendothelial system (liver, spleen, and lymph nodes). The involvement of other parenchymatous organs is rare, and there are only sporadic reports concerning the kidney. [1],[2],[3],[4],[5],[6],[7],[8] We describe a case of histologically documented EMH presenting as a solitary renal mass and mimicking a malignant tumor of the kidney in a patient with a known history of congenital dyserythropoetic anemia.


  Case Report Top


An 18-year-old male, diagnosed with congenital dyserythropoetic anemia type 1, presented with pain and swelling in the abdomen. Swelling was palpable in the right hypochondrium. Spleen was also moderately enlarged. Patient had history of multiple blood transfusions. Two siblings of the patient had already died because of this rare hematological disorder. CECT abdomen showed mass measuring 6.5 × 5 × 3.5 cm noted in lower pole of right kidney with mild homogenous contrast enhancement [Figure 1]. Moderate splenomegaly with dense area of calcification and prominent splenic vein. Gall bladder wall was thickened with cholelithiasis and choledocholithiasis. CT-guided biopsy was taken from the renal mass, but the material was inadequate for any definitive opinion. In view of doubtful renal mass, patient was admitted and planned for surgery. Intra-operatively, spleen was moderately enlarged. Since our patient had history of multiple transfusions, splenectomy was done to minimize the frequency of transfusions. Gallbladder was contracted and thick-walled containing multiple stones with a single stone in cystic duct. Kidney was enlarged, adherent superiorly to diaphragm and medially to duodenum. Huge lobulated hemorrhagic tumor was seen measuring 6.5 × 6 × 5 cm arising from lower pole of kidney [Figure 2]. Unfortunately, nephrectomy was done to remove the tumor en mass for final diagnosis. The histological examination revealed cells of three distinct lineages including myeloid and erythroid cells and few megakaryocytes in a hemorrhagic background [Figure 3]. Overall features were compatible with the final diagnosis of EMH presenting as a solitary renal mass. Foci of extramedullary hematopoiesis were seen in spleen also. Hilar lymph nodes of spleen were enlarged and showed reactive hyperplasia with sinus histiocytosis only.
Figure 1: CECT showing mass lesion in lower pole of right kidney with mild homogenous contrast enhancement

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Figure 2: Gross examination of nephrectomy specimen showing well circumscribed, lobulated hemorrhagic mass

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Figure 3: Microscopic examination reveals hematopoietic cells of all the three cell lineages. Three megakaryocytes seen in this field [Hematoxylin and Eosin, 400x]

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  Discussion Top


Development and growth of hematopoietic tissue outside of the bone marrow is termed extramedullary hematopoiesis (EMH). [7],[8] Although the particular process is essential in fetal life, its occurrence after birth is usually considered abnormal. As is the case with physiologic EMH in the fetus, the liver and spleen are the usual sites of pathologic EMH. However, non-hepatosplenic EMH (NHS-EMH) has been reported in a myriad of other tissues and organs, including the mediastinum, [9] central nervous system, [10] peripheral nerve, [11] middle ear, [12] pancreas, [13] urethra, [14] pharynx, [15] pleura and lungs, [16] pericardium, [17] heart, [18] gastrointestinal tract, [19] thyroid gland, [20] kidney, [18] prostate gland, [21] breast, [22] and endometrium. [23] Although NHS-EMH is often associated with myelofibrosis with myeloid metaplasia (MMM) [24] or thalassemia, [25] it can also accompany other disorders, including hereditary spherocytosis, [26] sickle cell anemia, [27] congenital dyserythropoietic anemia, [28] immune thrombocytopenic purpura, [29] chronic myeloid leukemia, [30] polycythemia vera, [31],[32] myelodysplastic syndrome, [33] Paget disease, [34] osteopetrosis, [35] and Gaucher disease [36] and treatment with myeloid growth factors. [37],[38],[39] Occasionally, an associated disease is not identified. [40],[41]

Congenital dyserythropoietic anemia type I (CDA I) is a rare autosomal-recessive disorder with ineffective erythropoiesis and iron overloading. Although many patients with CDA I are able to realize their social and professional goals, others may have relevant morbidity and if untreated, may even die of sequels related to CDA I. As in CDA II, 33 gallstones are detected more frequently and at younger age than expected in the healthy population. [42] Our patient was 18-years-old and had multiple stones in the gall bladder with one stone in cystic duct. Bulky extramedullary hematopoiesis may occur, and in our case, it was in one of the rarest sites-kidney.

The kidney is an unusual site for the occurrence of EMH, and clinically, renal EMH can be asymptomatic. There have been <20 previous reports of EMH renal involvement. [1],[2],[3],[4],[5],[6],[7],[8]

Renal involvement can be parenchymal, intrapelvic, or perirenal. In the parenchymal type, the kidneys may either be enlarged or have focal lesions and the masses may be indistinguishable from renal cell carcinoma. [2],[3] Pelvicalyceal or hilar involvement is often an extension of a parenchymal lesion pattern, and in this site, the EMH tissue may cause obstructive renal failure. [4],[5] In the perirenal type, the soft tissue encases both kidneys. The bilateral perirenal localization of EMH may sometimes mimic a renal lymphoma. [6]

The differential diagnosis of a perirenal or parapelvic mass of uncertain etiology includes tumors, lymphomas, lipomatosis and renal inflammatory or infectious tissue. [2],[6] Our patient presented with a solitary renal mass and was parenchymal and highly vascular.

In our case, we chose a CT-guided biopsy approach to arrive at a final diagnosis. But, no definitive opinion could be made on the scanty material, so nephrectomy was done to remove tumor en mass for definitive opinion.

Iron overloading has been recognized as a potential consequence in most but not in all patients and on long-term follow-up iron overloading with potential tissue damage is to be expected in almost all patients. [42],[43] Lifelong monitoring of serum ferritin is, therefore, mandatory, and iron depletion should be started if ferritin concentrations approach a level of 1000 μg/L, or if there is other evidence for organ damage by secondary hemochromatosis. Splenectomy is not recommended as a standard procedure, even in patients with marked anemia, although in exceptionally severe cases, it may lessen the need for transfusions. [43],[44],[45],[46] Our patient had history of multiple transfusions, so splenectomy was done to minimize the frequency of transfusions and iron overload.


  Conclusion Top


EMH presenting as a solitary renal mass is extremely rare, but it doesn't mean we should ignore its possibility. CT-guided biopsy should be the first-line investigation so that surgical intervention could be prevented in such cases. It should be always kept in mind before going for any radical procedure as was done in this case unfortunately.

 
  References Top

1.Ahuja S, Grover G, Jha AK, Sodhi K, Bansal D, Dey P. Extramedullary hematopoiesis presented as solitary renal mass: A case report with review of literature. Diagn Cytopathol 2010;39:435-7.  Back to cited text no. 1
[PUBMED]    
2.Gupta R, Seith A, Guglani B. Renal hilar extramedullary hematopoiesis presenting as incidental unilateral renal hilar mass in adolescent male: A case report. Int Urol Nephrol 2009;41:19-21.  Back to cited text no. 2
[PUBMED]    
3.Sairsorn I, Leewansangtong S, Sukpanichnant S, Ruchutrakool T, Leemanont P. Intrarenal extramedullary hematopoiesis as a renal mass in a patient with thalassemia. J Urol 2001;165:507-8.  Back to cited text no. 3
    
4.Woodward N, Ancliffe P, Griffiths MH, Cohen S. Renal myelofibrosis: An unusual cause of renal impairment. Nephrol Dial Transplant 2000;15:257-8.  Back to cited text no. 4
[PUBMED]    
5.Gibbins J, Pankhurst T, Murray J, McCafferty I, Baiden-Amissahk K, Shafeek S, et al. Extramedullary haematopoiesis in the kidney: A case report and review of literature. Clin Lab Haematol 2005;27:391-4.  Back to cited text no. 5
[PUBMED]    
6.Galperin-Aizenberg M, Volchek Y, Even Sapir E, Vasserman M, Maizlin ZV, Cohenpour M, et al. Renal extramedullary haematopoiesis mimicking renal lymphoma on computed tomography. Clin Radiol 2006;61:896-8.  Back to cited text no. 6
[PUBMED]    
7.La Fianza A, Torretta L, Spinazzola A. Extramedullary hematopoiesis in chronic myelofibrosis encasing the pelvicalyceal system and perirenal spaces: CT findings. Urol Int 2005;75:281-4.  Back to cited text no. 7
[PUBMED]    
8.Talmon GA. Pure erythropoiesis in clear cell renal cell carcinoma. Int J Surg Pathol 2010;18:544-6.  Back to cited text no. 8
[PUBMED]    
9.Guizzetti P. Haemolytischer kongenitaler Ikterus. Beitr Z Path Anat U Z Allg Path 1911;52:15-50.  Back to cited text no. 9
    
10.Cameron WR, Ronnert M, Brun A. Extramedullary hematopoiesis of CNS in postpolycythemic myeloid metaplasia [letter]. N Engl J Med 1981;305:765.  Back to cited text no. 10
[PUBMED]    
11.Gutsell RS. An anomalous case of blood formation in the connective tissue of the sciatic nerve in man. Anat Rec 1917;13:409-17.  Back to cited text no. 11
    
12.Applebaum EL, Frankel A. Extramedullary hematopoiesis of the middle ear. Am J Otolaryngol 1989;10:287-90.  Back to cited text no. 12
[PUBMED]    
13.Crider S, Kroszer-Hamati A, Krishnan K. Isolated pancreatic extramedullary hematopoiesis. Acta Haematol 1998;99:38-40.  Back to cited text no. 13
[PUBMED]    
14.Balogh K, O'Hara CJ. Myeloid metaplasia masquerading as a urethral caruncle. J Urol 1986;135:789-90.  Back to cited text no. 14
[PUBMED]    
15.Barnes EA, Seikaly H, Puttagunta L, Coupland RW, Jha N. Subglottic stenosis secondary to extramedullary hematopoiesis in a patient with postpolycythemia myeloid metaplasia. Head Neck 2000;22:435-8.  Back to cited text no. 15
[PUBMED]    
16.Ruebner B. Myelosclerosis with serosal myeloid metaplasia and fatal liver involvement. Ann Intern Med 1960;53:1075-88.  Back to cited text no. 16
[PUBMED]    
17.Shih LY, Lin FC, Kuo TT. Cutaneous and pericardial extramedullary hematopoiesis with cardiac tamponade in chronic myeloid leukemia. Am J Clin Pathol 1988;89:693-7.  Back to cited text no. 17
[PUBMED]    
18.Hill DA, Swanson PE. Myocardial extramedullary hematopoiesis: A clinicopathologic study. Mod Pathol 2000;13:779-87.  Back to cited text no. 18
[PUBMED]    
19.Gomes AS, Harell GS. Tumefactive extramedullary hematopoiesis of the stomach. Gastrointest Radiol 1976;1:163-5.  Back to cited text no. 19
[PUBMED]    
20.Fassina A, Fedeli U, Borsato S. Extramedullary hematopoiesis of the thyroid gland diagnosed by FNA cytology: A case report. Acta Cytol 1999;43:1181-3.  Back to cited text no. 20
[PUBMED]    
21.Humphrey PA, Vollmer RT. Extramedullary hematopoiesis in the prostate. Am J Surg Pathol 1991;15:486-90.  Back to cited text no. 21
[PUBMED]    
22.Malberger E, Hazzani A, Lemberg S. Extramedullary hematopoiesis in breast aspirates. Acta Cytol 1988;32:835-7.  Back to cited text no. 22
[PUBMED]    
23.Valeri RM, Ibrahim N, Sheaff MT. Extramedullary hematopoiesis in the endometrium. Int J Gynecol Pathol 2002;21:178-81.  Back to cited text no. 23
[PUBMED]    
24.Glew RH, Haese WH, McIntyre PA. Myeloid metaplasia with myelofibrosis: The clinical spectrum of extramedullary hematopoiesis and tumor formation. Johns Hopkins Med J 1973;132:253-70.  Back to cited text no. 24
[PUBMED]    
25.Korsten J, Grossman H, Winchester PH, Canale VC. Extramedullary hematopoiesis in patients with thalassemia anemia. Radiology 1970;95:257-63.  Back to cited text no. 25
[PUBMED]    
26.Bastion Y, Coiffier B, Felman P, Assouline D, Tigaud JD, Espinouse D, et al. Massive mediastinal extramedullary hematopoiesis in hereditary spherocytosis: A case report. Am J Hematol 1990;35:263-5.  Back to cited text no. 26
[PUBMED]    
27.Lewkow LM, Shah I. Sickle cell anemia and epidural extramedullary hematopoiesis. Am J Med 1984;76:748-51.  Back to cited text no. 27
[PUBMED]    
28.Lugassy G, Michaeli J, Harats N, Libson E, Rachmilewitz EA. Paravertebral extramedullary hematopoiesis associated with improvement of anemia in congenital dyserythropoietic anemia type II. Am J Hematol 1986;22:295-300.  Back to cited text no. 28
[PUBMED]    
29.Sharma AK. Tumefactive extramedullary hematopoiesis of the kidney in a patient with idiopathic thrombocytopenic purpura. AJR Am J Roentgenol 1996;167:795-6.  Back to cited text no. 29
[PUBMED]    
30.Gowitt GT, Zaatari GS. Bronchial extramedullary hematopoiesis preceding chronic myelogenous leukemia. Hum Pathol 1985;16:1069-71.  Back to cited text no. 30
[PUBMED]    
31.Rice GP, Assis LJ, Barr RM, Ebers GC. Extramedullary hematopoiesis and spinal cord compression complicating polycythemia rubra vera. Ann Neurol 1980;7:81-4.  Back to cited text no. 31
[PUBMED]    
32.de Morais JC, Spector N, Lavrado FP, Nobre LF, de Mattos JP, Pulcheri W, et al. Spinal cord compression due to extramedullary hematopoiesis in the proliferative phase of polycythemia vera. Acta Haematol. 1996;96:242-4.  Back to cited text no. 32
    
33.Dibbern DA Jr, Loevner LA, Lieberman AP, Salhany KE, Freese A, Marcotte PJ. MR of thoracic cord compression caused by epidural extramedullary hematopoiesis in myelodysplastic syndrome. AJNR Am J Neuroradiol 1997;18:363-6.  Back to cited text no. 33
[PUBMED]    
34.Kadir S, Kalisher L, Schiller AL. Extramedullary hematopoiesis in Paget's disease of bone. AJR Am J Roentgenol 1977;129:493-5.  Back to cited text no. 34
[PUBMED]    
35.Adams BK, Smuts NA. The detection of extramedullary hematopoiesis in a patient with osteopetrosis. Eur J Nucl Med 1989;15:803-4.  Back to cited text no. 35
[PUBMED]    
36.Ch'en IY, Lynch DA, Shroyer KR, Schwarz MI. Gaucher's disease: An unusual cause of intrathoracic extramedullary hematopoiesis. Chest 1993;104:1923-4.  Back to cited text no. 36
[PUBMED]    
37.Litam PP, Friedman HD, Loughran TP Jr. Splenic extramedullary hematopoiesis in a patient receiving intermittently administered granulocyte colony-stimulating factor. Ann Intern Med 1993;118:954-5.  Back to cited text no. 37
[PUBMED]    
38.Redmond J III, Kantor RS, Auerbach HE, Spiritos MD, Moore JT. Extramedullary hematopoiesis during therapy with granulocyte colony-stimulating factor. Arch Pathol Lab Med 1994;118:1014- 5.  Back to cited text no. 38
    
39.Neiman RS. Extramedullary hematopoiesis during therapy with granulocyte colony-stimulating factor [letter]. Arch Pathol Lab Med 1995;119:679-80.  Back to cited text no. 39
[PUBMED]    
40.Catinella FP, Boyd AD, Spencer FC. Intrathoracic extramedullary hematopoiesis simulating anterior mediastinal tumor. J Thorac Cardiovasc Surg 1985;89:580-4.  Back to cited text no. 40
[PUBMED]    
41.Elbers H, vd Stadt J, Wagenaar SS. Tumor-simulating thoracic extramedullary hematopoiesis. Ann Thorac Surg 1980;30:584-7.  Back to cited text no. 41
[PUBMED]    
42.Wickramasinghe SN, Thein SL, Srichairatanakool S, Porter JB. Determinants of iron status and bilirubin levels in congenital dyserythropoietic anaemia type I. Br J Haematol 1999;107:522-5.  Back to cited text no. 42
[PUBMED]    
43.Maeda K, Saeed SM, Rebuck JW, Monto RW. Type I dyserythropoietic anemia: A 30-year follow-up. Am J Clin Pathol 1980;73:433-8.  Back to cited text no. 43
[PUBMED]    
44.Holmberg L, Jansson L, Rausing A, Henriksson P. Type I congenital dyserythropoietic anaemia with myelopoietic abnormalities and hand malformations. Scand J Haematol 1978;21:72-9.  Back to cited text no. 44
[PUBMED]    
45.Bulky extramedullary hematopoiesis is not a rare complication of congenital dyserythropoietic anemia. Ann Hematol 2009;88:937-41.  Back to cited text no. 45
    
46.Choudhry VP, Saraya AK, Kasturi J, Rath PK. Congenital dyserythropoietic anaemias: Splenectomy as a mode of therapy. Acta Haematol 1981;66:195-201.  Back to cited text no. 46
[PUBMED]    


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