[C-reactive protein in differential diagnosis of primary thrombocytosis]. / La proteína C reactiva en el diagnóstico diferencial de la trombocitosis primaria.

BACKGROUND: The most potent stimulator for the hepatic synthesis of C-reactive protein is the interleukin-6. Also interleukin-6 is endowed with thrombopoietic activity, and its seric levels increases in most of secondary thrombocytosis whereas they remain normal in chronic myeloproliferative diseases or primary thrombocytosis. The aims of the study were verify the ability of quantitation of serum C-reactive protein in the differential diagnosis of primary thrombocytosis. METHODS: Serum samples from 89 patients with thrombocytosis (> 400 x 10(9)/1) and 54 normal controls were assayed for C-reactive protein. Patients with thrombocytosis were classified in primary thrombocytosis with 27 patients (chronic myeloproliferative disease with thrombocytosis) and secondary thrombocytosis (62 cases). RESULTS: The mean C-reactive protein serum levels observed in the 27 patients with primary thrombocytosis were 13 +/- 10 mg/l, superior to normal controls (7 +/- 5 mg/l; p < 0.01). In the secondary thrombocytosis group, C-reactive protein serum levels reached a mean value of 59 +/- 34 mg/l, clearly superior to control group and the primary thrombocytosis group (p < 0.0001). No patients in primary thrombocytosis group reached a C-reactive protein value > 40 mg/l, versus 65% of patients in secondary thrombocytosis group. A normal value occurred in 67% cases of primary thrombocytosis group, but also in 17% cases of secondary thrombocytosis group. CONCLUSIONS: Quantitation of C-reactive protein could thus prove useful in the differential diagnosis between primary and secondary thrombocytosis.

Familial myeloproliferative syndrome.

Familial chronic myeloproliferative syndrome (CMS) was observed in five members from two different generations of the same kindred. Diagnosis included agnogenic myeloid metaplasia (case 1), polycythemia vera (case 2), and essential thrombocythemia (cases 3-5). Cases 1-3 were siblings, case 5 was the daughter of case 1, and case 4 was the cousin of cases 1, 3. Age at diagnosis ranged from 28 to 75 years, cases 1 and 3 were male, and the others were female. The diagnosis was made after an episode of cerebral thrombosis in one patient, during a study for headache and dizziness in another, and fortuitously in the three remainders. All patients had splenomegaly and varying degrees of thrombocytosis. The cytogenetic exam was normal in all four cases. A woman patient was treated with interferon during a pregnancy. Fetal growth was retarded, and the newborn showed bone and genital malformations. No environmental leukemogen factor was found. This familial case strengthens Dameshek's theory of a common pathogenesis of CMS and suggests a genetic and hereditary etiology.

[Association of sarcoidosis, diffuse centroblastic-centrocytic lymphoma and IgM monoclonal gammapathy in the same female patient]. / Asociación de sarcoidosis, linfoma centroblástico-centrocítico difuso y gammapatía monoclonal IgM en una misma paciente.

A case illustrating the infrequent association of sarcoidosis and lymphoma which was also a secretor of a monoclonal IgM paraprotein is described in a 68 year old woman. The first entity manifested by dyspnea corresponded with a bilateral interstitial pneumopathy with restrictive syndrome and was diagnosed by bronchoalveolar lavage and transbronchial biopsy and biopsy prescalenic fat. The lymphoma presented three years later followed by lymphadenopathies and a monoclonal IgM peak being diagnosed by biopsy of an axillary lymph node. In order to relate both entities it has been suggested that prolonged stimulation of the lymphocytes peculiar to the sarcoidosis would cause its malignant transformation.