Hairy Cell Leukemia: A Reversible Disease?; A Report of Two Cases of Spontaneous Remission

Abstract

We report a case of a 27-year-old man with Ph1 positive chronic myelogenous leukaemia (CML). At the time of diagnosis 100% Ph1 positive cells were found with a trisomy 8 in 50% of them. In absence of therapy, his haematological status remained stable for 3 years. Subsequently a progressive regression of haematologic and cytogenetic data was observed. Eight years after diagnosis the karyotype showed only 37% Ph1 positive cells and the trisomy 8 had disappeared.

Case Details

Personal Characteristics

53-year-old male. This patient experienced several episodes of upper respiratory tract infections during his early years. Since the age of thirty he attempted periodic anti-inflammatory treatments for rheumatic pains but he never recovered completely.

Clinical Characteristics

In 1967, he was hospitalized for severe weakness, pallor and persistent headache. He was found to have marked anemia, leukopenia, liver and spleen enlargement. The bone marrow was nearly inaspirable: among the few cells obtained a significant number of atypical lymphoid elements with characteristic hairy-like cytoplasmic projections was detected. Surgical bone marrow biopsy revealed massive replacement by a population of the same type of cells and a diffuse stromal reaction with large amounts of reticulin. This condition was diagnosed as lymphoid myelofibrosis after duhamel (successively confirmed as typical hcl).

Remission Characteristics

Afterwards, he showed progressive reduction of the hepatosplenomegaly and an improvement of the haematologic picture so that no further treatment was given. Subsequently the patient resumed work and was followed up with periodic blood counts. In 1980 he underwent transurethral prostatectomy for prostatic hypertrophy. In december 1983, the patient was admitted for a re-evaluation of the case. Spleen and liver were no longer palpable; a trephine bone marrow biopsy revealed complete disappearance of lymphoid replacement and absence of fibrosis. In december 1984 he was still doing well without treatment 17 years after the diagnosis of hcl.

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

The patient was treated with blood transfusions, androgen's and prednisone for his moderate thrombocytopenia. In the following three years he had several hospital admissions due to recurrent infections, such as bronchitis, cystitis and cholecystitis, and to persistent mild anemia.

Additional Notes

Two out of these five patients never received any specific treatment. The aim of this communication is to report these two cases of spontaneous remission in HCL.