Clinico-haematological profile of patients with bicytopenia.

Bicytopenia is the reduction of any of the two cell lines of blood, i.e., erythrocytes, leukocytes or platelets. Many studies are done on pancytopenia but very few studies exist in the literature evaluating the spectrum of aetiologies of bicytopenia. To date, no study is available on bicytopenia in adults. We aimed to study the clinico-haematological profile of patients with bicytopenia and to investigate the different aetiologies of bicytopenia. Four hundred patients with bicytopenia admitted to KLE Dr. Prabhakar Kore Charitable Hospital and Medical Research Centre were selected using systematic random sampling and included in the study. Their clinical profiles and haematological parameters were evaluated. Bicytopenia was observed in all ages with the mean age being 30.7 years. The occurrence of bicytopenia in different age groups was 6% in neonates, 7% in infants, 25% in children, 17% in teenagers, 85% in adults and 11% in elderly. The most common bicytopenia observed was anaemia with thrombocytopenia (61%) followed by anaemia with leukopenia (26%) and leukopenia with thrombocytopenia (13%). The male to female ratio was 1.6:1. The most common aetiology of bicytopenia was found to be non-malignant (56%) followed by infectious (31.7%), malignant (8.3%) and drug-induced (4%). Megaloblastic anaemia was the predominant aetiology among the non-malignant group, closely followed by immune thrombocytopenic purpura and alcoholic liver disease. The most common infectious disease was dengue (12%). Signs like lymphadenopathy, splenomegaly and hepatomegaly were most significantly associated with haematological malignancies (p < 0.001). Pallor, bleeding, hepatomegaly and splenomegaly were most frequent in non-malignant conditions (p < 0.001). Fever and lymphadenopathy were most frequent in the infectious category (p < 0.001). The most prevalent signs in drug-induced aetiology were lymphadenopathy, hepatomegaly and splenomegaly (p < 0.001). Bicytopenia is a good haematological indicator for many non-malignant and malignant diseases. Knowledge of its aetiologies can help in the diagnosis and efficient management of patients.

Craniorachischisis Totalis with Congenital Diaphragmatic Hernia-A Rare Presentation of Fryns Syndrome.

Fryns syndrome is a multiple congenital anomaly syndrome with an autosomal recessive inheritance. Here we describe the autopsy case findings of a 19-week male fetus, born out of a consanguineous marriage. The dissection revealed left-sided diaphragmatic hernia, resulting in pulmonary hypoplasia and shift of heart to the right side. In addition, anencephaly and spina bifida throughout the vertebral column were observed. All six criteria for Fryns syndrome were met. Such a presentation of Fryns syndrome associated with Craniorachischisis Totalis has not been reported so far. We have also tabulated the overlapping features of some multiple congenital anomaly syndromes that need to be distinguished at autopsy for an accurate diagnosis.