HCL Meeting 2010
clinical picture of fulminating hepatitis and heman- giomata of the liver resulting from the large angiomatous lakes seen in the organs of the reticuloendothelial system in HCL [6,7,16,17]. At autopsy almost all cases of HCL were found to have liver involvement, and the true clinical prevalence of this site of disease is clearly underestimated [4] (Table IV).
Autoimmune disorders and hairy cell leukemia
HCL can also be associated with stigmata of autoimmune disease and with autoimmune disor- ders. The first cases were reported by Hughes in 1979 in association with polyarteritis nodosa and rheumatoid arthritis [18,19]. A larger series of cases with vasculitis was reported later in 1995 by Hasler [20], but the incidence in different series varies [6,7,21]. These can occur at any stage of the disease apparently, but at least half appear to develop within the first year after diagnosis. Vasculitis due to HCL infiltration may also be seen [20]. Most of the associated disorders are listed in Table V.
Associated rare infections and fever in hairy cell leukemia
Infection is a frequent complication of HCL because of the common combination of monocytopenia, neutropenia, decrease in the numbers of dendritic cells, and altered T-cell function [3,6,7,22–25]. The vast majority of patients (about 70%) will have infectious episodes [3,6,7,26], mostly with common
Table IV. Liver involvement.
. Diffuse pattern in advanced disease . Isolated tumorous nodules . Large cavernous hemangiomata: macro- and microscopic angiomatous lakes (in organs of RES, mostly liver and spleen)
. Clinically resembling ‘fulminant hepatitis’ RES, reticuloendothelial system.
Table V. Associations with autoimmune stigmata and disorders.
. Vasculitis ‘leukocytoclastic type’ . Lupus anticoagulants . Temporal arteritis . Cryoglobulinemia . Glomerulonephritis
. Autoimmune hemolytic anemia . Pernicious anemia . ITP-like
. Myasthenia gravis . Thyroiditis
. Rheumatoid arthritis
. Scleroderma, polymyositis . Periarteritis nodosa
. Ulcerative colitis . Amyloidosis
ITP, idiopathic thrombocytopenic purpura; CREST, calcinosis, Raynaud syndrome, esophageal dysmotility, sclerodactyly, telan- giectasia.
. Anticardiolipin syndrome . CREST
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pathogens, but uncommon opportunistic infections do occur and this should always be remembered, as they are more frequent with disease progression [26,27] and patients can present with opportunistic infection. The list of infections is given in Table VI. Infection with atypical mycobacterial disease [23,28,29] should always be considered in the differential diagnosis of ‘fever of unknown origin’ (FUO) in HCL, and even in patients with advanced disease who have organ involvement, splenomegaly, and obvious lymphadenopathy. Patients with com- bined infiltration with atypical mycobacterial disease and coexistent HCL have also been seen, very rarely.
Hairy cell leukemia coexistent and associated with solid tumors, hematological disorders, and hematopoietic neoplasias
A number of reports have implied an increased association of solid cancers in patients with HCL [30–32], and these have been summarized by Van Den Neste et al. [6] and others in other shorter reviews [7]. This has been debated in earlier studies from the Golomb group in 1985 and 1994 [31,32] and by Kurzrock et al. in 1997 [30]. This issue still remains controversial today, but there appears to be no true excess in the incidence of tumors beyond the expected prevalence. Clusters of cases of renal carcinoma, colon carcinoma, and skin tumors may indeed show a borderline increase, but this is both uncertain and unlikely. These are all listed in Table VII as are the hematological disorders and neoplasias reported to be associated with HCL .It appears that of all the hematological neoplasias reported, only myeloma and lymphoma are perhaps truly more prevalent in HCL [33–35]. Monoclonal gammopathy can occur in HCL (about 16% in one series) [36]; however, this is a relatively uncommon association [36–38]. Patients with other myeloproliferative dis- orders, such as polycythemia vera, myelofibrosis, myelodysplastic syndromes, leukemias, and Hodgkin lymphoma, have also been reported in HCL, but these associations probably have no true statistical significance.
Table VI. Opportunistic Infections.
. Atypical mycobacterial disease (kansasii, avium) . Candidiasis . Listeria, legionella . Pneumocystis . Cryptocococcosis . Toxoplasmosis . Aspergillosis . Sporotrix . Skin and lymph node infections . Rhizomucor
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