Indian Journal of Pathology and Microbiology

LETTER TO EDITOR
Year
: 2011  |  Volume : 54  |  Issue : 3  |  Page : 659--660

Bone marrow involvement in systemic oxalosis: A rare cause of leukoerythroblastic anemia


Naveen Kakkar1, Divya Mittal1, Sheila Das1, Joseph M John2, Timothy Rajamanickam3,  
1 Department of Pathology, Christian Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Clinical Hematology Unit, Christian Medical College and Hospital, Ludhiana, Punjab, India
3 Department of Nephrology, Christian Medical College and Hospital, Ludhiana, Punjab, India

Correspondence Address:
Naveen Kakkar
Department of Pathology, Christian Medical College and Hospital, Brown Road, Ludhiana - 141 008, Punjab
India




How to cite this article:
Kakkar N, Mittal D, Das S, John JM, Rajamanickam T. Bone marrow involvement in systemic oxalosis: A rare cause of leukoerythroblastic anemia.Indian J Pathol Microbiol 2011;54:659-660


How to cite this URL:
Kakkar N, Mittal D, Das S, John JM, Rajamanickam T. Bone marrow involvement in systemic oxalosis: A rare cause of leukoerythroblastic anemia. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 May 20 ];54:659-660
Available from: http://www.ijpmonline.org/text.asp?2011/54/3/659/85145


Full Text

Sir,

Leukoerythroblastic anemia is seen in patients with megaloblastic anemia, septicemia, acute hemolysis, hypersplenism, disseminated intravascular coagulation, myelofibrosis or bone marrow infiltrative disorders. [1] We present a patient with a rare cause of leukoerythroblastic anemia which was first diagnosed on bone marrow examination.

A 30-year-old woman presented with fatigue and vomiting on and off since one month. She was diagnosed to have chronic renal failure two years ago and was on regular hemodialysis. An ultrasonogram showed only one visualized shrunken echogenic kidney with extensive calcification. There was no history of joint pains or family history of similar illness. She had developed refractoriness to erythropoietin injections since the last two months.

On examination, she was pale. Spleen was palpable 5 cm below the left costal margin. There was no jaundice, lymphadenopathy or hepatomegaly.

Complete blood count showed hemoglobin of 5.2 gm/dl, total leukocyte count of 3800/ mm3 and normal platelet count. Differential leukocyte count and peripheral blood smear showed leukoerythroblastic anemia. Biochemical tests showed raised blood urea (103 mg/dl), serum creatinine (6.9 mg%) and lactate dehydrogenase (717 IU/l). Liver function tests and serum uric acid was normal.

Bone marrow aspiration was a dry tap with poorly cellular touch smears. The trephine biopsy showed replacement of the marrow space by radially arranged aggregates of grey-yellow crystals [Figure 1] with surrounding fibrosis, few ill-defined granulomas and scanty residual hematopoietic tissue. The crystals were birefringent when viewed under polarized light.{Figure 1}

A diagnosis of systemic primary oxalosis was made in view of characteristic morphology of crystals, renal involvement and no dietary history favoring secondary oxalosis. She continued hemodialysis for 6 months following diagnosis and was then lost to follow up.

Systemic oxalosis can be primary or secondary. Primary hyperoxaluria Type 1 (PH 1) is due to the deficiency of glyoxylate aminotransferase and Type II due to that of glyoxylate reductase/D-glycerate dehydrogenase. PH 1 is an autosomal recessive disorder characterized by hyperoxaluria, calcium oxalate urinary lithiasis in childhood, nephrocalcinosis and renal failure which in turn leads to high blood oxalate levels and precipitation occurs throughout the body in the skin, blood vessels and joints. Secondary oxalosis occurs due to oxalate-rich diet, increased absorption or production of oxalate and reduced excretion as seen in renal failure. Early stage of oxalosis is asymptomatic and diagnosis is often delayed and sometimes made incidentally. [2] Variable degree of cytopenia or pancytopenia may accompany extensive oxalosis. Bone marrow aspirate may be unsuccessful due to extensive crystal deposition or accompanying fibrosis. Calcium oxalate crystals are well demonstrated on trephine biopsy with associated fibrosis and granulomas. [3] They have a grey-yellow radial arrangement on hematoxylin and eosin staining and are birefringent under polarized light. Renal function tests are often deranged in patients with accompanying renal failure. Liver function tests may be abnormal in advanced disease. Liver biopsy to demonstrate reduced enzyme activity is the confirmatory test. Molecular diagnosis, being non-invasive, is preferred if available. [4] Long-term survival in patients is possible only with combined renal and hepatic transplantation. [5]

To conclude, the possibility of systemic oxalosis must be considered in a young patient with unexplained renal failure, splenomegaly and leukoerythroblastic anemia.

References

1Mehta AB, Hoffbrand AV. Haematological aspects of systemic disease. In: Hoffbrand AV, Catovsky D, Tuddenham EG, editors. Postgraduate haematology. 5th ed. Oxford: Blackwell Publishing; 2005. p. 965-78.
2Toussaint C, De Pauw L. Primary oxaluria. Nephrologie 1995;16:399-406.
3Walter MJ, Dang CV. Pancytopenia secondary to oxalosis in a 23-year-old woman. Blood 1998;91:4394.
4Halil O, Farringdon K. Oxalosis: an unusual cause of leucoerythroblastic anemia. Br J Haematol 2003;122:2.
5Cochat P, Gaulier JM, Koch Nogueira PC, Feber J, Jamieson NV, Rolland MO, et al. Combined liver-kidney transplantation in primary hyperoxaluria type 1. Eur J Pediatr 1999;158: S75-S80.