1/390. Chronic urticaria as a presenting sign of hairy cell leukemia. Chronic urticaria is a common clinical disorder that is idiopathic in over 75% of cases. Less commonly, urticaria may be the presenting manifestation of an allergic or infectious disease, endocrinopathy, inherited syndrome, or autoimmune disorder. Rarely, urticaria may be a sign of underlying malignancy, including leukemia. C.C. is a 48-year-old white female who was referred for evaluation of recurrent urticaria for 3 years. The pruritic, erythematous wheals were pinpoint, and appeared to be precipitated by heat, stress, and effort. Prick tests were negative except to D. pteronyssinus. CBCs over the past 5 years revealed WBCs of 2,300-5,000 cells/mm3. skin biopsy revealed interstitial edema with infiltration of eosinophils and mast cells consistent with urticaria. The impression was probable cholinergic urticaria, for which hydroxyzine was prescribed with fair symptomatic control. One year later, she presented with bright red blood per rectum. Repeat physical examination revealed lymphadenopathy and splenomegaly. Subsequent laboratory studies showed pancytopenia. endoscopy was normal except for small, nonbleeding hemorrhoids. bone marrow biopsy revealed histologic evidence of hair, cell leukemia that was treated with 2-chlorodeoxyadenosine. Upon initiation of chemotherapy her pruritus and urticaria subsided. Recent CBC revealed Hgb 9.2 g/dL, platelets 290,000 cells/mm3, and WBC 4,100 cells/mm3. Peripheral blood smear showed no hairy cells. ( info) |
2/390. Hairy cell leukemia with an associated lupus-type anticoagulant. Hairy cell leukemia is a rare, lymphoproliferative disorder usually of B-cell origin. It affects men more often than women by a ratio of 4:1, with approximately 600 cases per year in the united states. Leukemias in general are known to be associated with coagulopathies; however, the case reported here represents only the fourth reported case of hairy cell leukemia with an associated lupus anticoagulant. The lupus anticoagulant is unusual in that it is not truly a coagulation-preventing entity, but rather may result in a hypercoagulable state. The nomenclature for this manifestation derives from its ability to interfere with in vitro phospholipid-dependent tests of coagulation. This report includes a review and discussion of the case presented, as well as a review of both hairy cell leukemia and the lupus anticoagulant. ( info) |
3/390. Multiple basal cell carcinomas associated with hairy cell leukaemia. We report the case of a caucasian woman who, between the ages of 49 and 51 years, developed multiple (> 20) basal cell carcinomas (BCC). There was no family history of BCC. No abnormalities in the human homologue of the drosophila segment polarity gene patched (PTCH), glutathione S-transferases T1 and M1, or cytochrome P450 1A1 were detected by polymerase chain reaction (PCR)-based molecular analysis. There was, however, actinic damage of the skin in sun-exposed areas. The patient was diagnosed as having hairy cell leukaemia (HCL) at the age of 51 years, based upon leucocyte morphology as assessed by light and electron microscopy, tartrate-resistant acid leucocyte phosphatase (TRAP) staining, fluorescence activated cell scanning of peripheral blood leucocytes and bone marrow histology. As the leukaemia slowly progressed over a 3-month period, the patient developed four further BCCs. Given that HCL is characterized by a profound defect in T-cell function, it is conceivable that T-cell immune dysregulation can contribute to the pathogenesis of BCC, possibly enhancing the aetiological effect of ultraviolet irradiation. ( info) |
| A case of postoperative left chylothorax in a 43-year-old black woman with hairy cell leukemia is reported. First submitted to pleural drainage, she was successfully treated with a combination of chemotherapy and elemental enteral diet enriched with medium-chain triglycerides. ( info) |
5/390. Cryocrystalglobulinemia in hairy cell leukemia. A patient with hairy cell leukemia (leukemic reticuloendotheliosis) was noted to have a spurious leukocytosis caused by a spontaneously crystallizing cryoglobulin. The cryoprotein was identified as IgG lambda. An intracytoplasmic immunoglobulin demonstrable within the hairy cell was also IgG lambda. The cryoglobulin spontaneously disappeared over a four day period. A reliable automated count on the Coulter Model S could be obtained by prewarming the blood specimen to 37 C. ( info) |
6/390. An otherwise typical case of non-Japanese hairy cell leukemia with CD10 and CDw75 expression: response to cladaribine phosphate therapy. Hairy cell leukemia (HCL) in Western patients typically expresses CD19, CD20, CD11c, CD25, HLA-DR, and IgG/lambda and lacks expression of CD5 and CD10. The immunophenotype is in contrast to Japanese HCL which typically expresses CD5 and CD10. Western and Japanese HCL also differ in their clinical presentation and response to treatment with alpha-interferon. We report a case of non-Japanese HCL which presented typically with pancytopenia; however, the immunophenotype was atypical with expression of CD10 and CDw75. CDw75 expression has not previously been described in either Japanese or non-Japanese HCL. The patient achieved a marked partial pathologic response and complete clinical response to treatment with cladaribine phosphate. ( info) |
7/390. Acute febrile neutrophilic dermatosis (Sweet's syndrome) in hairy cell leukemia: a case report. Sweet's syndrome is a cutaneous reactive process that is usually associated with fever, but rarely occurs in patients with hairy cell leukemia (HCL). We report the case of a patient with HCL who developed typical Sweet's syndrome five months after the diagnosis of HCL. skin eruptions and constitutional symptoms subsided rapidly after short-term systemic adrenocorticosteroid treatment without recurrence, until the patient died from disease exacerbation and uncontrolled infection. According to his manifestations, chemical allergens, microorganisms or HCL progression were unlikely to have been the precipitating factors for development of Sweet's syndrome. Because immunologic disorders and opportunistic infection are not uncommon in patients with HCL, a skin biopsy should be taken as early as possible when cutaneous lesions and fever develop in order to establish a correct diagnosis. Hence, unnecessary and prolonged use of antibiotics is avoided and prompt relief of the symptoms by appropriate management can be achieved. ( info) |
8/390. Remission of psoriasis after treatment with interferon-alfa and 2-chlordeoxyadenosine for hairy cell leukemia. 2-Chlordeoxyadenosine (2-CdA) is an antineoplastic/immunosuppressive agent used to treat hairy cell leukemia (HCL), chronic lymphocytic leukemia, and low-grade lymphomas. Its immunomodulatory properties, however, may allow its future use in the treatment of psoriasis. We report a patient with psoriasis and HCL who was treated for 1 week with continuous intravenous infusion of 2-CdA for recurring HCL. Both the psoriasis and the HCL cleared. Four years after 2-CdA treatment, the patient has psoriasis on only 1% of his body surface area. 2-CdA induces lymphocytopenia, which may explain the improvement in this patient's psoriasis. ( info) |
9/390. Secondary acute myeloid leukemia 4 years after the diagnosis of hairy cell leukemia: case report and review of the literature. A 49-year-old man diagnosed with hairy cell leukemia (HCL) achieved a complete remission lasting 4 years after treatment with cladrabine and subsequently developed acute myeloid leukemia. Although a wide variety of second malignancies have been noted in HCL with an incidence of 8.7%, acute myeloid leukemia (AML) has been reported only once previously in a splenectomized patient who had been treated with alpha interferon. ( info) |
10/390. Hairy cell leukemia with translocation (11;20)(q13;q11) and overexpression of cyclin d1. We report on a male Japanese patient with hairy cell leukemia (HCL). A cytogenetic study with lipopolysaccharide stimuli showed a novel translocation (11;20)(q13;q11) in 10% of the analyzed cells. Northern blot analysis and RT-PCR analysis for cyclin d1 revealed the overexpression of cyclin d1, although the southern blot analysis of PRAD1 gene showed no rearrangement. In this particular case, the t(11;20)(q13;q11) might play some role in the oncogenesis of HCL and the overexpression of cyclin d1. ( info) |