RESUMEN
That the suggested criteria for diagnosis of polycythaemia vera (PV) are rather complicated, therefore accurate diagnosis of PV from other types of polycythaemia is needed. Serum Vitamin B12 and Vitamin B12 binding proteins were determined in a patients with PV and other 5 patients with secondary polycythaemia. Both serum Vitamin B12 and unsaturated Vitamin B12 binding capacity (UBBC) increased considerably. TCIII and TCI increased also markedly, i.e., 16 and 8 folds, respectively, resulting in a decreased ratio of TCI/TCIII from the normal value of 1.34 to 0.71. Patients with secondary polycythaemia had slightly increased serum Vitamin B12 while serum UBBC, TCI and TCIII levels showed no significant alteration from those of normal subjects. These findings indicated that not only increased serum Vitamin B12 and UBBC but also an increase of TCIII and the ratio of TCIII/TCI are the positive findings in patients with PV. Therefore determinations of serum Vitamin B12 and its binding proteins can be used as a criteria for the differential diagnosis of PV from secondary polythaemia.
RESUMEN
An 80-year-old woman was admitted to the hospital with of pain in the right upper abdominal region. She had splenomegaly and CT scan showed gall stones and splenic infarction. Blood examination revealed erythrocytosis, raised LAP, elevated serum vitamin B12, vitamin B12 binding proteins and reversed TCI/TCIII ratio. All these findings confirmed the diagnosis of polycythaemia vera. After splenectomy and cholecystectomy, a leucoerythroblastic blood picture with teardrop poikilocytosis was detected in the peripheral blood smear. The haemoglobin concentration decreased but leucocytosis and thrombocytosis still persisted. Bone marrow aspirate showed erythroid hypoplasia, decreased megakaryocytes and some degree of fibrosis. This was compatible with the criterial diagnosis of myelofibrosis with myeloid metaplasia. Blood transfusions and myleran were given. Peritoneal dialysis was performed and the patient had severe bleeding and died from cardiac failure due to septic shock. This patient is an example of the transition from polycythaemia vera to myelofibrosis with myeloid metaplasia.
RESUMEN
A 79-yesr-old woman was admitted to the hospital with evidence of pure red cell aplasia and chronic neutrophilic leukemia. Bone marrow aspiration showed almost absence of erythropoiesis, marked hyperplastic granulopoiesis with normal maturation and normal megakaryocytes. Serum vitamin B12, unsaturated vitamin B12 binding capacity, transcobalamin I and transcobamin III increased considerably. Chronic myelocytic leukaemia could be ruled out by the blood and bone marrow examination and a high leucocyte alkaline phosphatase score. This case of pure red cell aplasia accompanied by proliferative feature in other cell lines is an example of a link between erythrodysplastic and myeloproliferative disorders.
RESUMEN
An 83-year-old man with evidence of multiple myelome was admitted to the hospital with a history of weakness. His serum protein immunoelectrophoresis demonstrated monoclonal IgA lambda and bone marrow aspiration revealed plasma cells 20%. Two months alter, hematological examinations showed erythrocytosis, luecocytosis and thrombocytosis. The bone marrow showed hypercellular with increased plasma cells and monoblasts. Serum vitamin B12 and vitamin B12 binding proteins confirmed the diagnosis of polycythemia vera. After treatment with Busulfan, Prednisolone and Endoxan for 6 months, his blood examination revealed increased monocytes, metamyelocytes and myelocytes. Bone marrow examination confirmed the diagnosis of acute myelomonocytic leukemia with plasma cells. This patient is an example of the simultaneous occurrence of lympoproliferative and myeoproliferative disorder.