TESTS IN FOCUS
STORIES AT A GLANCE
Juvenile Hyaline Fibromatosis
Dr Alka Rani, Dr Madiha Bilal Qureshi and Dr Nasir Ud Din, Histopathology
Case Presentation
Seven year girl presented with multiple swellings on scalp, nose, gingival hypertrophy and ear swelling. 
Excision was done and multiple skin covered nodular tissue pieces were received ranging in size from 1.9- 4.5cm. Cut surface was grey, white homogeneous. The lesion showed variable cellularity with plump to spindled uniform fibroblasts, arranged in cords imparting vessels- like appearance. There was abundant eosinophilic non-fibrillar hyalinelike extracellular matrix. Immunostains ASMA, Desmin, CD34, S100, EMA and CD117 were negative. The features were diagnostic of Juvenile hyaline fibromatosis. Hyaline fibromatosis syndrome is an extremely rare autosomal recessive syndrome that typically affects infants, and causes painful, disfiguring, abnormal deposits of hyalinized matrix in the dermis, subcutaneous tissues and gingiva. Treatment is supportive with multiple excisions, recurrences are common.
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Indicators of Severe Malarial Infection
Dr Komal Masroor, Hematology
The timely diagnosis of malaria is crucial for preventing disease progression and reducing its severity. The major contributors to the
severity of malaria are anemia, cytopenias and leukocytosis. Presence of malarial pigment ‘hemozin’ (highlighted by red arrow) which is a by-product of parasite metabolism inside neutrophils and monocytes, affect host innate and inflammatory immune responses and inhibits erythropoiesis causing anemia. The presence of schizonts in a peripheral blood smear (highlighted by green arrow) is a sign of a high parasite burden increasing the likelihood of severe malarial infection.
