Hairy cell leukemia: bone marrow changes following splenectomy and alpha-interferon therapy.

Thirty hairy cell leukemia patients were evaluated repeatedly for their bone marrow (BM) histology. At the time of diagnosis, 18 (60%) had diffuse, 9 (30%) had interstitial, and 2 (10%) had a mixed (diffuse and interstitial) pattern of BM disease. The follow-up BM specimens were obtained at intervals of 3-24 months, and the follow-up observation period was 12-94 months. In patients who were nontreated or only splenectomized, no significant changes were observed except of a persistent megaloblastoid picture of the red cell series and an increase of BM fibrosis. In the alpha-interferon treated patients a complete disappearance of hairy cells was observed in one and a dramatic reduction in five. The hairy cell index was reduced from a mean of 0.8 before to 0.1 after alpha-interferon therapy; most patients displayed megaloblastoid erythropoiesis. In the complete responder features of myelodysplasia were present.

B-chronic lymphocytic leukemia. Prognostic implication of bone marrow histology in 120 patients experience from a single hematology unit.

The available staging systems for B-chronic lymphocytic leukemia (B-CLL) do not always predict the clinical course and the prognosis of the disease. In these systems, the pattern of bone marrow histology is not incorporated. In the current report we investigate the prognostic value of the diffuse or nondiffuse pattern of bone marrow involvement in 120 B-CLL patients in relation to their actuarial survival, and we compare these results with the actuarial survival based on the International Workshop system. In addition, we analyze the influence of the diffuse or nondiffuse pattern on the actuarial survival, in relation to the individual clinical stages (A, B, C). All patients were diagnosed and followed-up in the same Unit. Our patients were divided into Stage A (64), Stage B (22), and Stage C (34). They were also subdivided into those with a diffuse (46) and those with a nondiffuse (74) pattern of bone marrow histology. The difference in the actuarial survival in relation to their clinical stage (A, B, C) was statistically significant (P less than 0.025). A greater statistical difference (P less than 0.005) was found when the actuarial survival was analyzed in relation to the diffuse or nondiffuse pattern of bone marrow histology. No statistically significant differences could be found (P greater than 0.1), when the actuarial survival was calculated in every stage (A, B, C), on the basis of the diffuse or nondiffuse pattern of bone marrow histology. When our Stage A and B patients were analyzed for disease progression, in relation to the diffuse or nondiffuse bone marrow histology, it was found that 66.6% of the diffuse Stage A patients and 88% of the diffuse Stage B patients had disease progression as compared to only 8.6% for the nondiffuse Stage A patients and 33% for the nondiffuse Stage B patients. Our findings indicate that: the pattern of bone marrow histology in B-CLL patients is the single most important prognostic parameter in this disease; a clinicopathologic staging system for B-CLL may be justified; and the diffuse pattern of bone marrow histology could be considered as the best criterion for initiation of therapy in these patients.

Hemoglobin Knossos: a clinical, laboratory, and epidemiological study.

Hb Knossos is a beta-chain variant (beta 27 Ser----Ala) that is unrecognizable by conventional separation methods but detectable by globin electrophoresis on urea-Triton X-acrylamide gels or by IEF. Hb Knossos is characterized by reduced synthesis and by interaction with beta-thalassemia, in which the double heterozygotes display typical features of thalassemia intermedia. The present paper summarizes the salient genetic, clinical, and biochemical characteristics of five such cases hitherto identified in three families along with the same features on 12 heterozygous Hb Knossos carriers. Hb Knossos displays a slightly decreased oxygen affinity; this factor may compensate in part for the severe anemia of the double heterozygotes. Hb Knossos is relatively rare in our population, since a prospective survey on 610 individuals has failed to disclose any heterozygotes. However, the mutation appears to have spread over the Mediterranean countries and may be more common elsewhere.

Monoclonal and oligoclonal immunoglobulins in the serum of patients with B-chronic lymphocytic leukemia.

The incidence of monoclonal and oligoclonal immunoglobulins (paraproteins) was determined in serum samples of 45 chronic lymphocytic leukemia (B-CLL) patients using the high-resolution agarose gel electrophoresis technique combined with immunofixation. Paraproteins were identified in 25 of the 45 patients tested. Twelve paraproteins were monoclonal and 13 oligoclonal. IgG kappa and/or lambda immunoglobulin isotypes were found in 17/25 of these patients. No correlation of the lymphocyte cell morphology and the presence of paraproteins was demonstrated. The high frequency of serum oligoclonal immunoglobulins in B-CLL indicates that more than one lymphocyte clone may be present in this disease.