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November 1987, Volume 37, Issue 11

Case Reports

A CASE OF HB-SC DISEASE PRESENTING AS PRIAPISM

Noor Ahmad Noor  ( Department of Medicine, Nishtar Medical College, Multan. )
A.W. Qazi  ( Department of Medicine, Nishtar Medical College, Multan. )
Mohyuddin  ( Department of Medicine, Nishtar Medical College, Multan. )
Masood  ( Department of Medicine, Nishtar Medical College, Multan. )
Zulfiqar Ali  ( Department of Medicine, Nishtar Medical College, Multan. )

Priapism is a common symptom in Haemo­globin SC and SS disease, which results from thrombosis in the vascular spaces in the cavernous tissue of penis. Thrombosis is due to altered physico-chemical properties of HbS which has glutamic acid substituted for valine in the sixth position from N-terminal in Beta Chain. When deoxygenated, HbS is less soluble and tactoids form in red cells which cause distortion and damage to the red cell membrane. Sickled red cells become rigid and mechanically fragile resulting in haemolysis, repeated vascular occlusions and infarction of various interbak organs like kidneys, mesentery, intestine, lungs, brain and bones.

CASE REPORT

A 30 years old Balochi farmer from Loralai was admitted with priapism of 7 days duration. There was no history of dysuria, haematuria or trauma. On clinical examination he was slightly pale; spleen, liver, lymph nodes and kidneys were not palpable and there was no other abnormality. On investigation, Hb-l0.2 G/dl; total and differential leucocyte count, reticulocyte count, platelet count and blood urea were normal. The peripheral blood did not show any malarial parasite. When a drop of blood was sealed under a cover slip on a slide, red cells acquired sickle shape after 24 hours. Sodium metabisulphate test was not done. Paper electrophoresis revealed a distinct band of HbS and C.

DISCUSSION

In this rare case, HbSC was responsible for priapism. Other cases of priapism described are leukemias (both myeloid and lymphatic), new growth of urethera, primary and secondary carcinoma of bladder or testes, sickle cell anaemia, trauma to penis, or injury to upper dorsal region of spinal cord1.
Frequent priapism, sexual impotence and reduced fertility has been reported as a compli­cation in sicklers from African subcontinent2.
Neuropsychiatric symptoms like delerium and temporal lobe epilepsy or grand mal epilepsy may also be seen3. Sudden, unexpected deaths are not uncommon in SS or SC disease and are due to superimposed bacterial, viral or malarial infec­tions4. The mothers with SC disease behave normally during pregnancy5.
Treatment is mainly conservative in the form of use of sedatives or analgesics and correction of fluid and electrolyte balance.
The case reported above was treated on conservative lines and his priapism was relieved within a week.

REFERENCES

1. Hart, RD. ed. French’s index of differential diagnosis. 1 ithed. London, John Wright, 1979, p.657.
2. Osegbe, D.N., Akinyanju, 0. and Amaku, E.O. Fertility in males in sickle cell disease. Lancet, 1981; 2:275.
3. Ovuga, E.B.L. and Acuda, S.W. Neuropsychiatric disorders in sickle cell trait. East Mr. Med. J., 1986;62:730.
4. Williams, A.O. Sickle cell disease and pathologists. East Afr. Med. J ., 1977 ; 54 :14.
5. Scebali, E.C.T. and Buiwa, F.H. Sickle cell trait anaemia in pregnancy. East Mr. Med. J., 1977; 54:258.

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