Assessment of the severity of hereditary spherocytosis using routine haematological data obtained with dual angle laser scattering cytometry

Dual angle laser scattering cytometry (DALC) measures erythrocyte volume and haemoglobin concentration directly and provides spherocyte quantification. We have studied DALC red cell features in hereditary spherocytosis (HS) relating them to the clinical severity of the illness. A total of 140 controls and 80 non-splenectomized HS cases were studied, with mild (n = 32), moderate (n = 37) and severe (n = 11) disease. DALC measured mean corpuscular haemoglobin concentration, percentage of erythrocytes with haemoglobin concentration over 410 g/l (%Hyper), red cell volume and haemoglobin concentration distribution widths were significantly different in HS cases when compared to controls (P < 0.001). Severe HS cases had more heterogenous erythrocyte populations, both in volume and haemoglobin concentration (P < 0.001). Clinical severity of HS was best reflected by erythrocyte volume distribution width followed by %Hyper. DALC red cell features are both a diagnostic tool and an index to the severity of HS.     

Cardiomyopathy and phaeochromocytoma

A case is presented of phaeochromocytoma with catecholamine-induced cardiomyopathy. The difficulties in the management when left ventricular failure occurred are reported and the importance of monitoring pulmonary artery pressure during the anaesthetic procedure is stressed.     

Pancreatic encephalopathy

A prospective study was conducted on 17 cases of acute pancreatitis; encephalopathy was discovered in six patients (35 %). The following parameters were studied: the usual ones in pancreatitis conditions (amylasemia, lipasemia, amylasuria, ions, glucose, _pO2, _pCO2, _pH, etc.), and electroencephalographic changes and determinations in CSF of cells, proteins, lipase, amylase, lipides and cholesterol.
A direct relationship was found to exist between the pancreatic encephalopathy condition and an increase in CSF-lipase. The electroencephalographic changes were nonspecific. The encephalopathy did not affect the course of the pancreatitis condition, and showed no relationship to type of treatment involved. The severity of the pancreatitis was not related to the presence or absence of encephalopathy.     

The effect of splenectomy in hereditary spherocytosis by dual angle laser light cytometry

Summary We have applied dual angle laser scattering cytometry (DALC), which provides objective assessment of spherocytosis, to study the changes of hereditary spherocytosis (HS) red cell populations after splenectomy. Eighty unsplenectomized HS patients (32 mild, 37 moderate and 11 severe cases), 26 splenectomized HS patients (SHS, formerly 21 moderate and five severe cases) and 140 controls were studied. SHS were similar to unsplenectomized mild HS cases, with homogeneous red cell volume distribution and spherocytosis. Spherocytosis in SHS was significantly higher when compared with controls (P < 0.001) but less when compared with unsplenectomized HS patients (P < 0.01).