Indian Journal of Pathology and Microbiology

LETTER TO EDITOR
Year
: 2012  |  Volume : 55  |  Issue : 3  |  Page : 423--424

Thrombocytopenia, giant platelets and Leukocyte inclusion bodies in a patient presenting with vaginal bleeding


Manujasri C Wimalachandra, Manurie G Prabashika, Lallindra V Gooneratne 
 Department of Pathology, Faculty of Medicine, University of Colombo, Sri Lanka

Correspondence Address:
Manujasri C Wimalachandra
Department of Pathology, Faculty of Medicine, 25, Kynsey Road, Colombo 08
Sri Lanka




How to cite this article:
Wimalachandra MC, Prabashika MG, Gooneratne LV. Thrombocytopenia, giant platelets and Leukocyte inclusion bodies in a patient presenting with vaginal bleeding.Indian J Pathol Microbiol 2012;55:423-424


How to cite this URL:
Wimalachandra MC, Prabashika MG, Gooneratne LV. Thrombocytopenia, giant platelets and Leukocyte inclusion bodies in a patient presenting with vaginal bleeding. Indian J Pathol Microbiol [serial online] 2012 [cited 2019 Oct 23 ];55:423-424
Available from: http://www.ijpmonline.org/text.asp?2012/55/3/423/101771


Full Text

Sir,

A 42 year old female presented with a history of heavy per vaginal bleeding following a period of amenorrhea and a subsequent diagnosis of hydatidiform mole was made. During the pre-operative work up she was found to have persistent thrombocytopenia (platelet count<30,000 x10 9 /l) with a high mean platelet volume (mean platelet volume 12.5 fl). She had no evidence of sepsis or other bleeding manifestations. Her systemic examination was unremarkable, with no lymph node enlargement or organomegaly.

A peripheral smear revealed giant hypergranular platelets [Figure 1] and basophilic inclusions in the granulocytes [Figure 2]. Light transmission platelet aggregometry was performed to exclude Bernard-Soulier syndrome. Normal aggregation was seen with adenosine diphosphate, arachidonic acid, epinephrine, ristocetin and collagen. Bone marrow aspiration revealed trilineage hemopoiesis with increased numbers of megakaryocytes.{Figure 1}{Figure 2}

The basophilic inclusion bodies which were also present in the granulocytes of the bone marrow were identified as Dφhle bodies. Dφhle bodies are small, round or oval, pale blue-grey structures usually found at the periphery of the neutrophil. [1] They occur in all types of leukocytes except lymphocytes. They are seen in a variety of acquired conditions such as infections and burns and a few inherited disorders such as May Hegglin anomaly (MHA), Fechtner syndrome (FS) and Sebastian syndrome (SS). [2] All three conditions have an autosomal dominant inheritance and present with the classical triad of thrombocytopenia, giant platelets and leukocyte inclusion bodies. They can be caused by a number of different mutations that occur within the MYH 9 gene located on the long arm of chromosome 22 which encodes the 224-kD non-muscle myosin heavy chain IIA (MYHIIA) polypeptide. [2]

MHA is by far the commonest among the inherited megathrombocytopenias mentioned above. Most patients are asymptomatic or present in adulthood with a mild bleeding tendency. [3] Rarely, they may have significant bleeding associated with childbirth or surgical procedures. Platelet transfusions are the only means of effective treatment. SS differs from MHA only by a difference in the ultrastructure of the inclusion bodies demonstrated by electron microscopy. FA can be distinguished from MHA and SS by the presence of Alport-like clinical features such as sensorineural deafness, cataracts and nephritis. [2]

This patient underwent suction evacuation under platelet cover and her post operative period was also complicated with heavy bleeding. Screening of her two children revealed that her son had the classical triad of MHA while her daughter did not.

References

1Lewis SM, Bain BJ, Bates I, editors. Dacie and Lewis: PRACTICAL HAEMATOLOGY. 10 th ed. Philadelphia: Churchchill Livingstone Elsevier; 2006.
2Seri M, Cusano R, Gangarossa S, Caridi G, Bordo D, Lo Nigro C et al. Mutations in MYH9 result in the May-Hegglin anomaly, and Fechtner and Sebastian syndromes. The May-Heggllin/Fechtner Syndrome Consortium. Nat Genet 2000;26:103-5.
3Noris P, Spedini P, Belletti S, Magrini U, Balduini CL. Thrombocytopenia, giant platelets, and leukocyte inclusion bodies (May-Hegglin anomaly): Clinical and laboratory findings. Am J Med 1998;104:355-60.