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Neutropenia, Agranulocytosis and Dipyrone

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Neutropenia, Agranulocytosis and Dipyrone Neutropenia, Nelson Hamerschlak Alexandre Biasi Cavalcanti agranulocytosis and dipyrone Instituto Israelita de Ensino e Pesquisa Albert Einstein, Oncology & Hematology Program, São Paulo, Brazil INVITED REVIEW INTRODUCTION 75% are in the storage pool, 3% in the mar- ABSTRACT Neutropenia may be defined as a neu- ginated pool and 2% in the circulating pool. Under normal conditions, 1.5 x 109 granulo- CONTEXT: Neutropenia and agranulocytosis trophil granulocyte count of less than 1,500/ may be defined as granulocyte counts of less 3 mm . This definition can be applied to any cytes/kg are produced per day. Inflammatory than 1,500/mm3 and 500/mm3, respectively. age group, although certain groups may be processes increase this production. The granu- Agranulocytosis is a rare and serious disease interpreted in relation to some particular locytes live for nine days in the bone marrow, often caused by drugs. Its mortality rate is around 10%. The most common manifestations characteristics. For instance, blacks and Ye- three to six hours in blood and one to four are infections such as tonsillitis, pharyngitis, meni Jews usually present lower granulocyte days in tissues. Therefore, it must be borne in stomatitis or pneumonia. Although dipyrone is count. Agranulocytosis may be defined as a mind that any interpretation of results should one of the drugs known to be associated with 3 agranulocytosis, the strength of the association neutrophil count of less than 500/mm . consider that measurements in the peripheral has been a matter of much debate. Moreover, The peripheral neutrophil count reflects blood represent a small share of neutrophils alternative analgesic and antipyretic agents are the balance between several compartments. In and a short period of useful life. not devoid of serious side effects. the bone marrow, there is the mitotic pool, the Neutropenia may occur because of CONCLUSIONS: It is therefore necessary to maturation pool and the storage pool. Outside decreased production of granulocytes, shift establish the incidence of agranulocytosis in of granulocytes from the circulating com- Latin America and the role of dipyrone. The on- the bone marrow, there are circulating pools, going LATIN Study is a multicenter international the marginated pool adhering to the vascular partment to the marginated or tissue pool, case-control study that will provide answers for endothelium and the tissue pool. Clinical peripheral destruction, or a combination of these questions. trials to establish the number of neutrophils these three mechanisms (Table 1).1 Many KEY WORDS: Agranulocytosis. Neutropenia. measure only the circulating pool in transit agents can cause neutropenia. The mechanism Dipyrone. Incidence. Case-control studies. from the bone marrow to tissues. The move- may directly involve the bone marrow, such as ment is usually made in the direction bone in the case of antineoplastic agents; be related marrow-blood-tissue. to formation of antibodies and complements The biology of hematopoiesis is complex against hematopoietic precursors; and, more and regulated by many cytokines. Some rarely, involve peripheral destruction with cytokines, such as G-CSF and GM-CSF neutrophil clearance. (granulocyte colony-stimulating factor and The main clinical manifestations of granulocyte-monocyte colony-stimulating agranulocytosis are fever, tonsillitis, pharyngi- factor), together with complementary com- tis, sepsis, stomatitis and pneumonia. As most ponents, are able to increase the release of physicians know, it is a rare event and its inci- granulocytes from the bone marrow storage dence ranges from 1.7 to 7.0 cases/million in- pool to circulation. This may result in a two to dividuals/year.2 The mortality rate is 10%, and threefold increase in the number of leukocytes complete recovery is expected in over 80% of within four to five hours. The marginated patients.3 With appropriate antibiotic therapy pool, representing over half the number of and bone marrow growth factors, today this granulocytes in peripheral blood, may also rate is tending to drop significantly. be released to circulation. Epinephrine is one of the main elements in this process, which Incidence and etiology explains the neutrophilic response to stress and of agranulocytosis – exercises. Many other mediators are involved, the LATIN Study such as L-selectin, P-selectin, lactoferrin and The LATIN Study started in Brazil and acid isoferritins. has been extended to other Latin American Out of a total of 1.2 x 109 granulocytes/kg, countries, since there were no appropriate data 20% are precursors of the bone marrow pool, on the incidence and risk factors of agranulo- Sao Paulo Med J. 2005;123(5):247-9. 248 cytosis and aplastic anemia in these countries. the incidence of agranulocytosis and aplastic The pilot study has already shown inter- Data restricted to Brazil on the incidence and anemia in Latin America and to assess the pos- esting results that were used for adjusting the risk factors of aplastic anemia were presented sible regional differences in the association of definite protocol. Seven centers took part in by Maluf et al.4 aplastic anemia and agranulocytosis with the the pilot study, representing the main regions The only data available on the incidence risk factors mentioned.6 in Brazil.6 Seventy-four cases of aplastic ane- of agranulocytosis in Brazil are from a study The eligibility criteria for agranulocyto- mia, 16 of agranulocytosis and 103 controls published in 1993. This is a retrospective sis were defined as granulocytes < 500/mm3 were enrolled. Although no definite conclu- evaluation of some cases of drug-induced with decreased granulocytic series in the sion may be drawn, since this was not the agranulocytosis in São Paulo, a city with marrow, or recovery of granulocyte count to purpose of the pilot study, the incidence was 12 million inhabitants, and it showed an normal values after 30 days; hemoglobin ≥ 0.5 cases/million individuals/year and 2.7 incidence of 0.44-0.82 cases/million in- 10 g/dl, platelets ≥ 100,000/mm3, and bone cases/million individuals/year for agranulocy- habitants/year.5 marrow study (when performed) ruling out tosis and aplastic anemia, respectively. The doubt about the use of medicines in other diagnoses.6 The LATIN Study is being carried out cases of agranulocytosis is related to knowing For case-control studies, the appropriate in four Latin American countries: Brazil, Ar- which drug caused the problem. The patients choice of controls is as important as the selec- gentina, Colombia and Mexico. The principal presenting this disease have usually taken more tion of cases. Therefore, the eligible controls hematologists and university professors in this than one drug. Which drug is involved: the had to have: field are taking part in it. The Advisory Board one the patient took yesterday or another • Been admitted within a three-month comprises well-known epidemiologists and taken seven days ago? In the case of analgesic interval, at most, and to the same hospital authors of several studies on the topic, includ- drugs or antipyretics, which came first, the or treatment center as the cases; ing Dr. Juan Laporte, Dr. David Kaufman chicken or the egg? Did the patient take an • �����������������������������������������Presented acute diseases, such as trauma, and Dr. Samuel Shapiro, who provide the antipyretic because he/she had agranulocytosis appendicitis or acute infections. Alter- necessary scientific support. or did the drug caused the problem? natively, patients admitted for surgical While still in its initial planning phase, The LATIN Study was designed to treatment of oligosymptomatic condi- the LATIN Study was presented at a meet- attempt to answer some questions about tions, such as cataract, hallux valgus, nasal ing held in the Ministry of �ealth entitled aplastic anemia and agranulocytosis by us- septum deviation and plastic surgery, were “Dipyrone efficacy and safety panel”.7 It was ing a case-control study with evaluation of accepted; concluded from this meeting that withdraw- incidence. The primary objective was to as- • ������������������������������������������ad any clinical conditions due to chronic ing dipyrone from the market would result sess the possible association of drugs, diseases disease complications ruled out; in increased indiscriminate use of other and environmental and occupational factors • ����������������������������������Been pair-matched for sex and age. analgesic and antipyretic agents that are not with agranulocytosis and aplastic anemia. exempt from side effects. The secondary objectives were to estimate The controls were excluded from the One of the recommendations of the study in the event of present or past histories panel was to follow-up the results from the Table 1. Causes of neutropenia of the following: LATIN Study, which would serve as a drug • �������������������������������������Use of chemotherapy, radiotherapy and surveillance system. Two reports have already Acquired neutropenia immunosuppressants; been sent to Anvisa (the Brazilian �ealth Post-infectious • ��������������������������������������ematological and neoplastic diseases; Surveillance Agency) since the panel was held; Drug-induced • �����������������������������������������Systemic diseases that are known to cause the most recent of these reports described the Benign familial neutropenia neutropenia or pancytopenia, such as lu- results from the pilot study. Benign chronic neutropenia in children pus, �IV infection and hypersplenism;
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