Artrite reumatoide do tipo EORA com trombocitose significativa: relato de caso / Elderly-onset rheumatoid arthritis with significant thrombocytosis: a case report

Este relato teve como objetivo apresentar um caso de elderly onset rheumatoid arthritis associada à trombocitose reacional significativa. À admissão, o paciente apresentava quadro de poliartrite de pequenas e grandes articulações associado à rigidez matinal. Após exames solicitados, evidenciaram-se trombocitose de 1.697.000 cel./mm³ e anticorpos antipeptídeos citrulinados positivos, sendo diagnosticado com artrite reumatoide do tipo elderly onset rheumatoid arthritis.

This report aimed at presenting a case of elderly-onset rheumatoid arthritis associated with significant reactive thrombocytosis. On admission, the patient presented polyarthritis of small and large joints associated with morning stiffness. After the performance of the requested tests, thrombocytosis of 1,697,000 cells/mm3 and positive anti-CCP were evidenced, and the patient was diagnosed with elderly-onset rheumatoid arthritis.

Clinicohematological study of thrombocytosis.

Objective. To find out etiology and clinical course of thrombocytosis in Indian pediatric population. Methods. A total of 250 patients having thrombocytosis (defined as platelet count >500 x 109/L) on haematological investigations were studied over one yr period. All patients were evaluated clinically and were subjected to investigations, including complete blood counts (CBC) with peripheral smear examination. To elucidate the possible role of inflammatory cytokines in pathogenesis of RT, levels of Interleukin-6 (IL-6) and C - reactive protein (CRP) were estimated. Results. Infants and young children (<2 yr age) were most common group, contributing 60% of total cases. Out of total 250 cases, only 3 (1.2%) cases were found to have primary thrombocytosis and remaining 98.8% cases were having RT. Among RT patients, infections (alone or in association with iron deficiency anemia) were most common cause, accounting for 65% cases, while iron deficiency anemia (IDA) was second most common cause accounting for 41.3% cases (12.6% IDA alone and 28.7 % in association with infections). Other causes included nutritional dimorphic anemia and patients on treatment for megaloblastic anemia, acute lymphoblastic leukemia (during treatment) and lymphoma. Among various groups of RT, IL-6 and CRP levels were higher in patients with infection with or without IDA than IDA alone. One child with essential thrombocytosis and one child with RT had thrombotic complications. On follow up, platelet counts normalized in most of the patients with treatment of underlying conditions. Conclusions. Results of this study suggest that essential thrombocytosis is extremely rare in children. Infections and IDA (alone or in association with infections) are common causes of RT. IL-6 and CRP levels are increased in patients with RT, to a higher level in patients with infections than in patients with IDA. Most patients with RT have uneventful recovery of platelet counts to normal range with treatment for underlying condition.

Hematological changes in tuberculous spondylitis patients at the Hospital Universiti Sains Malaysia.

In this study, we compared the platelet count with erythrocyte sedimentation rates (ESR) in patients with tuberculous spondylitis to evaluate the correlation. This was a retrospective 3-year study covering January 2004 to December 2006 at the Hospital Universiti Sains Malaysia. Platelet counts, hemoglobin levels, ESR, peripheral blood counts and peripheral blood smears on 17 patients with tuberculous spondylitis were obtained. The ages of the patients ranged from 20- to 70-years-old. The male to female ratio was 3.2:1. The majority of the patients were anemic (88.2%) and 52.9% of the patients had thrombocytosis. All the patients had normal lymphocyte counts and a high in ESR at diagnosis. There was a linear correlation between the platelet count and ESR (r = 0.60, p < 0.01). The platelet count was also significantly correlated with the hemoglobin level (r = -0.6, p < 0.02). The degree of thrombocytosis was related to the degree of inflammation measured by the ESR. Thrombocytosis also correlated with the hemoglobin level. We suggest that evaluating hematological values in suspected cases of tuberculosis should be considered. The presence of hematological changes should raise the suspicion of tuberculosis in spondylitis patients.

JAK2(V617F) Mutation in Korean Patients with Essential Thrombocythemia / 대한진단검사의학회지

BACKGROUND: Essential thrombocythemia (ET) is thought to reflect transformation of a multipotent hematopoietic stem cell, but its molecular pathogenesis remains obscure. But tyrosine kinase, especially Janus kinase 2 (JAK2), has been implicated in myeloproliferative disorders other than chronic myeloid leukemia. We investigated the frequency of JAK2 mutation and its correlation with other clinicopathologic variables in Korean patients with ET and reactive thrombocytosis (RT). METHODS: JAK2 mutation analysis was performed on genomic DNA from bone marrow aspirates of 24 patients with ET and peripheral blood in 36 patients with RT using allele-specific PCR. RESULTS: JAK2 mutation was detected in 11 patients (46%) among the 24 patients with ET and was not found in 36 patients with RT. In patients with ET, older age and leukocytosis were related with JAK2 mutation without statistical significance (P=0.172 and 0.094, respectively). But this mutation was not correlated with sex, hemoglobin, platelet count, splenomegaly, increased cellularity of bone marrow, bone marrow fibrosis and vascular complications. CONCLUSIONS: The current observation strengthens the specific association between JAK2 mutation and ET. At the diagnosis of ET in Korean patients, identification of JAK2 mutation should be incorporated in the basis for new approaches.

Platelet large cell ratio in the differential diagnosis of abnormal platelet counts.

Platelet volume indices, estimated by automated blood cell analyzers, are useful in the diagnosis of various conditions with abnormal platelet counts. Platelet count (PLT), Platelet large cell ratio (P-LCR), Platelet distribution width (PDW) and mean platelet volume (MPV) were studied in 779 patients with normal platelet counts, 74 patients with high platelet counts and 41 cases with low platelet counts. P-LCR was significantly decreased in patients with thrombocytosis than in normal while it was increased in thrombocytopenia. In patients with high counts, P-LCR was significantly decreased in reactive thrombocytosis than neoplastic thrombocytosis. P-LCR was increased in destructive thrombocytopenia than those with hypoproliferative thrombocytopenia though it was not statistically significant. P-LCR was inversely related to platelet count and directly related to PDW and MPV. Platelet large cell ratio if properly utilised can be a good aid in the differential diagnosis of conditions associated with abnormal platelet counts.

Superior mesenteric artery aneurysm presenting with epigastric pain and raised platelet count.

A 90-year-old man admitted to our hospital for recuperation after heart failure was found to have raised platelet count and a normocytic anemia. He then developed an acute abdomen and died. Post-mortem examination revealed ruptured superior mesenteric artery aneurysm. Reactive thrombocytosis and anemia were probably the only indication of

Trombocitosis en la edad pediátrica / Thrombocytosis in paedriatric patients

El valor normal de plaquetas varía entre 150,000 y 450,000 x mm al cubo. Se define trombocitosis como un recuento mayor de 600,000. Pueden ser primarias, por un trastorno mieloproliferativo o secundarias a un gran número de patologías. Objetivos: conocer en nuestro medio la frecuencia de ambos tipos de trombocitosis, describir las patologías asociadas y relacionar la magnitud de la trombocitosis con los diferentes diagnósticos. Material y métodos: se analizaron 18,000 hemogramas realizados entre enero y diciembre de 1998, en el Hospital Roberto del Río. Se evaluó sexo, recuento de plaquetas y leucocitos, hematocrito, hemoglobina, VCM, CHCM y diagnósticos. Resultados: se encontró trombocitosis en 584 exámenes (3,24 por ciento). Se evaluaron 334 fichas, el 62 por ciento eran de sexo masculino. El 0,9 por ciento presentó cifras de plaquetas > 1,000,000 x mm al cubo (dos casos fueron trombocitosis primarias: trombocitemia esencial y leucemia mieloide crónica y un caso de meningitis bacteriana). Las trombocitosis secundarias se asociaron a: infecciones (48,8 por ciento), principalmente respiratorias, deficiencia de hierro (18,6 por ciento) y daño tisular (12,6 por ciento). Conclusiones: la frecuencia de trombocitosis en niños es baja. Cuando la trombocitosis es menor de 1,000,000 x mm al cubo debe sospecharse una etiología secundaria

Serum C-reactive protein level in postsplenectomized thalassemic patients.

C-reactive protein is an established marker for the detection of acute and chronic inflammatory processes. The most potent stimulator for the hepatic synthesis of this protein is interleukin 6. Previous studies have shown that inflammatory cells and inflammatory cytokines, such as interleukin 6, interferon gamma, etc were elevated in postsplenectomized thalassemic patients. The aim of this study was to determine serum C-reactive protein concentration in postsplenectomized beta thalassemic patients (beta thal/HbE postsplenec), and to compare them with those in nonsplenectomized beta thalassemic patients (beta thal/HbE), postsplenectomized non thalassemic patients (postsplenec), reactive thrombocytosis (RT), chronic myeloproliferative disorders (MPD) and normal adult volunteers. Serum C-reactive protein concentration as determined by an automatic Behring Nephelometer was carried out in 28 beta thal/HbE postsplenec, 22 beta thal/HbE, 12 postsplenec, 23 RT, 21 MPD, and 26 healthy adult volunteers. The values of CRP in beta thal/HbE postsplenec were significantly higher when compared with beta thal/HbE, and normal volunteers (4.1 +/- 0.7 vs 1.6 +/- 0.4 mg/L P = 0.006, and 4.1 +/- 0.7 vs 0.45 +/- 0.09 mg/L, P < 0.001). CRP levels in beta thal/HbE postsplenec were also higher than the postsplenec group (4.1 +/- 0.7 vs 0.19 +/- 0.7 mg/L P = 0.095). On the contrary, they were significantly lower than those in RT (4.1 +/- 0.7 vs 55.4 +/- 14.8 mg/L, P = 0.002). However, when compared to those with MPD, the values were not statistically different (4.1 +/- 0.7 vs 17.1 +/- 12.3 mg/L, P = 0.871). Interestingly, there was a trend towards increasing C-reactive protein levels in beta thal/HbE postsplenec patients with higher platelet count, although no correlation was observed. Besides the inflammatory process, platelet and/or factor(s) that control(s) thrombopoiesis seem(s) to play a role in the high serum C-reactive protein levels in the studied population.

Platelet counts in thalassemic children before and after splenectomy.

Even though thrombotic risks in thalassemia patients from standpoints of platelet dysfunction and coagulation factors are controversial, they are in favor of thrombosis due to thrombocytosis. From the study of 74 cases of thalassemia in children, marked thrombocytosis occurred during day 8 to 4 months during which one should be aware of the thrombosis. However, none of thalassemia children had acute thrombosis even at platelet counts of 1.6 million/microliters.

Trombositosis en pacientes con linfoma / Thrombocytosis in lymphoma patients

La trombocitosis asociada a linfoma ha sido descrita en forma muy esporádica, por esta razón hicimos un estudio retrospectivo en 59 pacientes con linfoma. En 24 (40%) se encontró al menos 2 determinaciones de plaquetas mayores de 450.000.mm 3, 13 (54%) con Enfermedad de Hodgkin (EH) y 11 (46%) con linfomas No-Hodgkin (LNH). La mayoría de los pacientes con LNH (72%) correspondían a linfomas de intermedio y alto grado de malignidad. El estadio se pudo precisar en el 75% de los pacientes y de éstos el 95% correspondían a estadios III o IV. En el 87% de los enfermos la trombocitosis coincidió con: actividad de la enfermedad 70%, infección 10%, actividad e infección 20%. Se concluye: 1.- La trombocitosis es relativamente frecuente durante la evolución de los pacientes con linfoma, 2.- Generalmente se encuentra en estadios avanzados, 3.- Se asocia a actividad de la enfermedad o infección

Trombocitose e doença de Crohn / Thrombocytosis and Crohn disease

A correlaçäo entre trombocitose e atividade da doença de Crohn (DC) tem sido constatada por alguns autores. Entretanto, esta observaçäo näo tem sido extensamente utilizada pela existência de comprovaçöes limitadas. Assim, os autores analisaram, retrospectivamente, os dados dos últimos 30 pacientes atendidos na Disciplina de Gastroenterologia do Departamento de Clínica Médica do Hospital das Clínicas da Faculdade de Medicina da Universidade de Säo Paulo, e na clínica privada de um dos autores (A.B.). Levou-se em consideraçäo a contagem de plaquetas (método de Brecher-Cronkite; valores normais 200.000 a 400.000 plaquetas/mm3) e o índice clínico de atividade da DC segundo os critérios de Harvey e Bradshaw. Onze pacientes (37%) apresentaram trombocitose (x=500.818 + ou - 56.845) e o valor médio do índice clínico de atividade desse grupo foi 8,6 (intervalo de confiança: 6-13). Dezenove pacientes (63%) näo apresentaram trombocitose (267.789 + ou - 62.124) e o seu valor médio de atividade foi 5,7 (intervalo de confiança: 2-11). Diferença estatisticamente significante foi verificada entre os valores médios dos índices clínicos de atividade dos dois grupos (z=2,84, para alfa < ou = 0,05), bem como entre as médias do número de plaquetas/mm3 (z = 4,5, para alfa < ou = 0,05). Näo houve relaçäo estatisticamente significante entre trombocitose e formas íleo-cólica, jejuno-ileal e cólica da DC. Entretanto, dos nove pacientes da casuística com forma ileal, oito (88%) näo apresentaram trombocitose e tiveram índices de atividade < ou = 6. Os possíveis mecanismos fisiopatológicos da trombocitose na DC säo comentados e revisados. A relaçäo entre trombocitose e a atividade da DC, observada na presente casuística, näo constitui, certamente, elemento diagnóstico para a afecçäo, mas associadamente ao quadro clínico, pode representar importante parâmentro para indicar a atividade da doença