Leukemia & Lymphoma, June 2011; 52(S2): 57–61
Unusual clinical manifestations, rare sites of involvement, and the association of other disorders with hairy cell leukemia
TAMAR TADMOR1 & AARON POLLIACK2
1Hematology Unit, Bnai-Zion Medical Center, Haifa, Israel and 2Department of Hematology, Hadassah University Hospital, Hebrew University Medical School, Jerusalem, Israel
Abstract Unusual clinical manifestations, rare sites of involvement, and associations with other disorders and malignancies occurring in patients with hairy cell leukemia (HCL) are uncommon events encountered in a relatively rare disease. The exact prevalence of these associations is difficult to determine accurately in HCL as they are often anecdotal case reports and not always detailed in all larger series of patients. This short review deals with the unusual clinical manifestations and rare sites of involvement of the disease and lists some of the disorders associated with HCL, based on what has been reported in the literature as well as from personal experience. No attempts are made here to establish the true prevalence of these phenomena and only selected references are included. Some details of the coexistence of HCL with other neoplasias, hematological disorders, and ‘paraneoplastic’ autoimmune disorders are provided, while opportunistic infections in HCL, particularly atypical mycobacterial disease, are briefly discussed. For the sake of brevity many of the details are provided in tabular form.
Keywords: Hairy cell leukemia (HCL), unusual manifestations, autoimmune disease, second cancer, opportunistic infections
Introduction Hairy cell leukemia (HCL) is a rare but distinct form of B-cell neoplasia, and therapy with purine analogs, such as cladribine and pentostatin, is very effective in achieving clinical responses and impressive complete remissions. The cell biology and pathogenesis of HCL are still not well understood but some recent advances have been made particularly in relation to the molecular biology of this disease. Conventional diagnosis is based on the typical clinical features, with varying degrees of pancytopenia and the presence of splenomegaly in the absence of lympha- denopathy in most cases, associated with the presence of HCL cells in the peripheral blood and bone marrow with increased marrow reticulin fibrosis. The characteristic ‘hairy’ cells are generally tartrate resistant acid phosphatase (TRAP) positive and have a specific immunophenotypic profile defined by flow cytometry [1,2]. Ultrastructural features of HCL are typical, and include the presence of typical ribosomal lamellae complexes on transmis- sion electron microscopy and a classsical complex surface architecture showing microvilli and ruffled folds evident on scanning electron microscopy. The
above combination of clinical findings and laboratory features is what would be considered the usual manifestations of HCL. Rarer patients display unusual clinical manifestations and rare sites of leukemic involvement, and infrequent associations with other systemic disorders and neoplasias occur. However, their true prevalence in HCL is difficult to determine accurately as many are described as anecdotal case reports and are not always featured in the larger series of patients. These are briefly outlined below.
Unusual clinical manifestations and rare sites of leukemic involvement
The exact prevalence of these rare and unusual presentations is difficult to determine, but they have been well documented. In 1987, in amost interesting publication, Bouroncle reported a cohort of 116 patients with HCL emphasizing rare sites of involve- ment and unusual presentations and complications, and shedding some light on the incidence of these uncommon findings [3]. Generally, unusual cases are reported in an anecdotal fashion as individual
Correspondence: Aaron Polliack, Department of Hematology, Hadassah University Hospital, Hebrew University Medical School, Jerusalem, Israel. E-mail:
apol@cc.huji.ac.il
ISSN 1042-8194 print/ISSN 1029-2403 online 2011 Informa UK, Ltd. DOI: 10.3109/10428194.2011.565395
Page 1 |
Page 2 |
Page 3 |
Page 4 |
Page 5 |
Page 6 |
Page 7 |
Page 8 |
Page 9 |
Page 10 |
Page 11 |
Page 12 |
Page 13 |
Page 14 |
Page 15 |
Page 16 |
Page 17 |
Page 18 |
Page 19 |
Page 20 |
Page 21 |
Page 22 |
Page 23 |
Page 24 |
Page 25 |
Page 26 |
Page 27 |
Page 28 |
Page 29 |
Page 30 |
Page 31 |
Page 32 |
Page 33 |
Page 34 |
Page 35 |
Page 36 |
Page 37 |
Page 38 |
Page 39 |
Page 40 |
Page 41 |
Page 42 |
Page 43 |
Page 44 |
Page 45 |
Page 46 |
Page 47 |
Page 48 |
Page 49 |
Page 50 |
Page 51 |
Page 52 |
Page 53 |
Page 54 |
Page 55 |
Page 56 |
Page 57 |
Page 58 |
Page 59 |
Page 60 |
Page 61 |
Page 62 |
Page 63 |
Page 64 |
Page 65 |
Page 66 |
Page 67 |
Page 68 |
Page 69 |
Page 70 |
Page 71 |
Page 72 |
Page 73 |
Page 74 |
Page 75 |
Page 76 |
Page 77 |
Page 78 |
Page 79 |
Page 80 |
Page 81 |
Page 82 |
Page 83 |
Page 84 |
Page 85 |
Page 86 |
Page 87 |
Page 88 |
Page 89 |
Page 90 |
Page 91 |
Page 92 |
Page 93 |
Page 94 |
Page 95 |
Page 96 |
Page 97 |
Page 98 |
Page 99 |
Page 100 |
Page 101 |
Page 102 |
Page 103 |
Page 104 |
Page 105 |
Page 106 |
Page 107 |
Page 108 |
Page 109 |
Page 110 |
Page 111 |
Page 112 |
Page 113 |
Page 114 |
Page 115 |
Page 116 |
Page 117 |
Page 118 |
Page 119 |
Page 120 |
Page 121 |
Page 122