VOLUME 32 | ISSUE 3 | 2012

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Fever of unknown origin, anemia and thrombocytosis as early symptoms and signs of a late-onset polymialgia rheumatica: a diagnostic challenge

Pasquale Niscola, Laura Scaramucci, Marco Giovannini, Andrea Tendas, Luca Cupelli, Alessio Pio Perroti, Paolo de Fabritiis

From the Hematology Unit, Saint Eugenio Hospital, Rome, Italy

How to cite this article:

Niscola P, Scaramucci L, Giovannini M, Tendas A, Cupelli L, Perroti AP, et al. Fever of unknown origin, anemia and thrombocytosis as early symptoms and signs of a late-onset polymialgia rheumatica: A diagnostic challenge. Ann Saudi Med 2012; 32(3):322.

To the Editor: Fever of unknown origin (FUO) associated with anemia and thrombocytosis may represent a diagnostic challenge for the hematologist. Indeed, a wide spectrum of disorders, including infections, collagen vascular diseases, solid tumors, and hematological malignancies should be considered in this situation. Moreover, although anemia and thrombocytosis in association with FUO may be compatible with some myeloproliferative disorders, this clinical framework may be the sign of an inflammatory or neoplastic disease.1 In addition, mild-to-moderate thrombocytosis may be encountered in patients with rheumatologic disorders,2,3 although the coexistence of this finding with anemia and FUO as early manifestations of a late-onset polymyalgia rheumatica (PMR) is an uncommon occurrence, so that the resulting clinical framework may represent a diagnostic challenge. We recently observed a 66-year-old female with fever, anorexia, weight loss, night sweats and depression. Laboratory examinations showed a worsening anemia along a progressive increasing of the platelet count. These findings were initially thought to be compatible with a slowly-resolving infectious mononucleosis. Two years before she was submitted to an endoscopic resection of a villous colorectal adenoma; apart from this finding, the past medical history was otherwise unremarkable. The physical examination was substantially uninformative; in particular, neither the abdominal organs nor the superficial lymph node was palpable; no physical signs for vasculitis, musculoskeletal and rheumatic diseases were found. A comprehensive work-up was performed. The hemogram showed a hemoglobin (Hb) concentration of 8.9 g/dL; a reduced count of normocytic and hypochromic red blood cells (RBC) and an increased platelet count (586x000/uL) were found. The iron serum level was slightly reduced whereas the ferritin concentration showed normal values. Nonspecific inflammatory tests, included erythrocyte sedimentation rate (120 mm/h) and C-reactive protein (CRP=15.4 mg/dL), were highly increased. Liver and renal functions were normal. Coagulation tests were normal, although elevated plasma levels of fibrinogen (634 mg/dL) and of D-dimer (350 ng/mL) were present. Antibodies against Epstein-Barr virus (EBV), cytomegalovirus, HIV1/2, and hepatitis A, B, C were negative. Urine and blood cultures were negative. Serum and urinary electrophoresis were negative; the urinalysis and examination of the urine sediment revealed no abnormalities. The serum levels of rheumatoid factor and specific autoantibodies associated with most frequent autoimmune diseases were normal. The morphological examination of smears from peripheral blood showed slightly hypocromic RBC, throbocytosis and platelet anisocytosis but not other significant abnormalities; in particular, lymphocytosis and atypical lymphocytes were not found. The radiological assessment included a chest radiography, a body computed tomography scan and an ultrasonography of the abdomen, which revealed no significant abnormalities; an echocardiography showed normal findings. A relapsing colorectal adenoma and other inflammatory and neoplastic bowel diseases were excluded by a pancolonoscopic examination; an esophagogastroduodenoscopy ruled out any mucosal abnormalities and Helicobacter pylori infection. The morphological examination of the bone marrow (BM) smears and of the trephine biopsy revealed a hyperplasia of megakaryocytic and erythroid lines; no atypical cells and no increasing of BM fibrosis were found. The iron content in the BM was normal. Cytogenetic evaluation of a BM sample revealed a normal kariotype; the absence of Janus kinase-2 (JAK-2) and Bcr-Abl mutations were demonstrated by molecular studies. This inexplicable clinical framework persisted throughout this diagnostic course which lasted for about two months when she presented with an aching and distressing pain afflicting the muscles of the neck and the shoulder; moreover, she complained of morning stiffness in the shoulder and neck. A prompt revaluation was soon made and the persistence of the pathological findings was confirmed; in particular, ESR (130 mm/h) and CRP (12.3 mg/dl) were highly elevated whereas hematological parameters were substantially stable. Radiological evaluation of the cervical spine and of shoulder and an orthopedic consultation revealed no abnormalities. A provisional diagnosis of PMR was made for which prednisone, at the starting dose of 20/day was given. The patient did not complain of visual impairment, headache nor signs and symptoms compatible with cerebrovascular complications of any type and origin; in addition, ophthalmology and neurology consultations revealed no abnormal findings. The patient refused a temporal artery biopsy for which a complicating giant cell arteritis (GCA) was excluded only on a clinical basis. The patient felt better a few hours after beginning the steroid medication; a complete pain relief was observed within three days whereas the laboratory abnormalities progressively ameliorated until the near-normalization observed after two weeks of prednisone. In the light to the prompt response to prednisone, the definite diagnosis of PMR was confirmed.4 To date, three months after the start of treatment, the patient is well and receiving 5 mg/day of prednisone, after a slow and careful tapering of the dose. In conclusion, our case showed that anemia and thrombocytosis may be associated with PMR although it represents an unusual occurrence which should be considered in the evaluation of patients referred for hematological consultation because of a chronic inflammatory state.

 

 

References

1. Harrison CN, Bareford D, Butt N, Campbell P, Conneally E, Drummond M, et al. Guideline for investigation and management of adults and children presenting with a thrombocytosis. Br J Haematol 2010;149:352-75.

2. Harten P, Seyfarth B, Löffler H. Thrombocytosis in polymyalgia rheumatica: An additional diagnostic criterion and possible risk factor for ischemic complications? Arch Intern Med 1998;158:95-7.

3. Ayvaz OC, Yavasoglu I, Kadikoylu G, Bozkurt G, Bolaman Z. Thrombocytosis in rheumatoid arthritis: JAK2V617F-positive essential thrombocythemia. Rheumatol Int 2011 [In Press].

4. Zimmermann-Górska I. Polymyalgia rheumatica: Clinical picture and principles of treatment. Pol Arch Med Wewn 2008;118:377-80.

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