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CASE REPORT
Year : 2012  |  Volume : 39  |  Issue : 2  |  Page : 100-101

Acquired pure red cell aplasia in children


1 Department of Pathology, Jawaharlal Nehru Medical College, KLE's University, Nehru Nagar, Belgaum, Karnataka, India
2 Department of Medicine, Jawaharlal Nehru Medical College, KLE's University, Nehru Nagar, Belgaum, Karnataka, India

Date of Web Publication1-Oct-2012

Correspondence Address:
Sujata R Dafale
Department of Pathology, Jawaharlal Nehru Medical College, KLE's University, Nehru Nagar, Belgaum - 590 010, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-5009.101862

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  Abstract 

Acquired Pure Red Cell Aplasia (PRCA) is a rare occurrence in children.This is a case of an eight year old girl child who developed acquired PRCA secondary to long term intake of sodium Valproate. This case is reported to review the causes of PRCA in children and to reconsider the use of drugs of longer duration in children and adults.

Keywords: Acquired pure red cell aplasia, children, sodium valproate


How to cite this article:
Dafale SR, Chavan RY, Hungund BR, Kalashetty M, Alatgi A C. Acquired pure red cell aplasia in children. J Sci Soc 2012;39:100-1

How to cite this URL:
Dafale SR, Chavan RY, Hungund BR, Kalashetty M, Alatgi A C. Acquired pure red cell aplasia in children. J Sci Soc [serial online] 2012 [cited 2018 Sep 19];39:100-1. Available from: http://www.jscisociety.com/text.asp?2012/39/2/100/101862


  Introduction Top


Pure Red Cell Aplasia (PRCA) is a rare condition of profound anemia characterized by the absence of reticulocytes and virtual Erythroblastopenia in the bone marrow. All other lineages are present and seem morphologically normal. It is of two types Congenital andand Acquired (Acute andand Chronic Type). The congenital form of the disease is known as the Diamond-Blackfan anemia, which is associated with other congenital abnormalities. [1],[2]


  Case Report Top


An eight year old girl child suffering from spastic quadriplegia with mental retardation since birth had history of repeated convulsions. Subsequently she was put on anticonvulsant therapy (sodium valproate) and is on anticonvulsant therapy for the last sevenseven years. She started developing pallor, breathlessness, weakness by fourfour years of age. There was no history of lump in abdomen, bleeding from any site, jaundice, bone pains, joint swellings or rash. There was no history of blood transfusions received by her family members any time in the past. There was no history of tuberculosis or contact with tuberculosis. Apart from pallor, her general examination findings were non-contributory. Cardiovascular examination revealed a functional systolic murmur at the base of the heart. Per-abdomen and Respiratory System were normal. Central nervous system examination revealed spastic quadriplegia.

Investigations done were Hb: 2.2gm%, PCV: 8.8%, Retic count: 0%, RBC count: 0.90 million/cmm, MCV: 93.3 fl, MCH: 31.8 pg, MCHC: 34.1%, and RDW 18.3%. Total WBC count: 5,200/cmm, Differential WBC count- Polymorphs: 59%, Lymphocytes: 35%, Eosinophils: 2%, and Monocytes: 4% and Platelet count: 2,08,000/cmm.

Peripheral smear showed normocytic, hypochromic red cells with occasional macrocytes and tear drop cells.

Bone marrow aspiration and trephine biopsy showed red cell hypoplasia, with markedly reduced erythroid precursors and normal myeloid and megakaryocytic series [Figure 1] and [Figure 2]. The M: E ratio was 12.2:112.2
Figure 1: Bone marrow aspirate showing markedly decreased Erythroid series with normal Myeloid and megakaryocytes (Leishman stain, ×400).

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Figure 2: Trephine biopsy showing markedly decreased Erythroid series with normal Myeloid and Megakaryocytes (H and E, ×400)

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Stool for occult blood: Negative. Serum bilirubin: 0.8 mg%. Hb F: 0.38%. Direct and indirect antiglobulin tests: Negative. Liver and renal function tests: Normal. Chest X-ray: Normal. Mantoux test: Negative. CT scan of the chest ruled out thymoma. Serum immunoglobulins revealed an IgG level of 1200 mg% (N - 565 to 1570 mg%), IgM of 87.5 mg% (N - 28 to 135 mg%) and IgA of 360 mg% (N - 102 to 545 mg %) respectively. Rheumatoid factor: Negative, Anti- nuclear antibodies andand anti-dsDNA: Negative. HIV and HbsAg: Negative.

The diagnosis of Acquired Pure red cell aplasia of chronic type was made and was due to long term intake of Sodium Valproate. The above investigations ruled out the other causes for acquired PRCA. The offending drug was stopped and the patient was put on other antiepileptic drug. After 4 weeks the Hb% increased steadily.


  Discussion Top


Kaznelson first described pure red cell aplasia, more than 70 years ago. [2] It occurs in age group between 7-70 years. It may be congenital or acquired. Congenital PRCA is also known as Diamond-Blackfan syndrome. The Incidence of Acquired PRCA is increasing. In the adult it is often idiopathic. Acquired primary PRCA commonly occurs at a mean age of 60 years. [2] Rare familial cases have been reported. The male to female ratio is 2:1. It has been postulated that primary PRCA has an autoimmune basis, which has been confirmed by in-vivo and in-vitro studies. The other causes are Thymoma, Autoimmune diseases and and Malignancies (Leukemia, Lymphoma, Multiple Myeloma, and Carcinomas of several organs), drugs (including alpha Interferons, Lamivudine, INH, Diphenylhydantoin etc.) and infections like tuberculosis. [2],[3],[4] PRCA may represent the prodrome to a Myelodysplastic syndrome (MDS). [2],[5]

Acquired PRCA is rare in children The commonest cause of Acquired PRCA of chronic type in children are Infections (Tuberculosis, B 19, Parvovirus infection and and HIV) followed by drugs, Leukemias, Hemolytic Anemias etc. Valproate, Anti Tubercular therapy, Diphenylhydantoin, Co-trimoxazole, Penicillin and Phenobarbitone are few of the drugs used for longer duration. [2],[6]

In the Congenital PRCA there is injury to the stem cells in utero. In Acquired PRCA there is virus or drug induced impairment of the cells that have differentiated from stem cells and can express erythropoietin receptors. Thus the damage is mainly immune andand T cell mediated. Thus stem cells are not the targets in Acquired PRCA. [7]

This case is reported to review the causes of PRCA in children and to reconsider the use of drugs of longer duration in children and adults.

 
  References Top

1.Frickhofen N, Chen J, Young NS, Cohen BJ, Heimpel H, Ambokowitz JL. Parvovirus as a cause of acquired pure red cell aplasia. Br J Haematol 1994;87:818-24.  Back to cited text no. 1
    
2.Sharma RA, Hiwarkar P, Manglani MV, Muralidhar HP. Acquired pure red cell aplasia in a child. J Postgrad Med 2002;48:37-8.  Back to cited text no. 2
[PUBMED]  Medknow Journal  
3.Maggi G, Casido C, Cianci R, Molinati M, Ruffini E. Thymoma: Results of 241 operated cases. Ann Thorac Surg 1991;51:152-6.  Back to cited text no. 3
    
4.Marseglia GL, Locatelli F. Isoniazid- induced pure red cell aplasia in two siblings. J Pediatrics 1998;132:898-900.  Back to cited text no. 4
[PUBMED]    
5.Dutta S, Mohta R, Pati HP. Tuberculosis and pure red cell aplasia. Int J Tuberc Lung Dis 1999;3:361-2.  Back to cited text no. 5
[PUBMED]    
6.Bunn HF. Drug-induced autoimmune red-cell aplasia. N Engl J Med 2002;346:522-3.   Back to cited text no. 6
[PUBMED]    
7.Charles RJ, Sabo KM, Kidd PG, Abkowitz JL. The Pathophysiology of pure red cell aplasia: Implications for therapy. Blood 1996;87:4831-8.  Back to cited text no. 7
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Abstract
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Case Report
Discussion
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