Purpose of reviewChediak-Higashi syndrome, a rare autosomal recessive disorder, was described over 50 years ago. Patients show hypopigmentation, recurrent infections, mild coagulation defects and varying neurologic problems. Treatment is bone marrow transplant, which is effective in treating the hem

Chediak-Higashi syndrome.

Purpose: To review the recent advances in the biologic and clinical research of chronic lymphocytic leukemia. Data Identification: English-language literature search using MEDLINE (1980 to 1990) an...

Chronic Lymphocytic Leukemia: New Insights into Biology and Therapy

The clinical and laboratory features of 300 patients with chronic lymphocytic leukaemia (CLL) and prolymphocytic leukaemia (PLL) of B-cell type were studied in order to investigate the relationship between these two diseases. Statistical analysis demonstrated that more than 55% circulating prolymphocytes (PROL) was a defining criterion for PLL, disorder characterized by marked splenomegaly without lymph-node enlargement, cells with high density of membrane-immunoglobulin (SmIg), low mouse-rosettes (M-rosettes) and strong reactivity with the monoclonal antibody FMC7. Patients with typical CLL, defined as having less than 10% PROL, were on average 10 years younger than those with PLL and showed preferential lymph-node to spleen involvement. Characteristic markers of CLL were weak SmIg, high M-rosettes and low reactivity with FMC7. Patients with 11-55% PROL, group designated as CLL/PL, were found to have intermediate features between CLL and PLL: the degree of splenomegaly was disproportionate to the lymph-node enlargement, the number of cases with strong SmIg was closer to that found in PLL, but the other markers were not significantly different from CLL. The CLL/PL group appeared to be heterogeneous and includes at least two types of CLL, one with increased proportions of PROL but otherwise typical disease, and another in 'prolymphocytoid' transformation. Our study suggests that although PLL cannot be considered as the extreme end of a continuous spectrum from typical CLL, the spleen may be the source of PROL both in PLL and in CLL/PL.

The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. I. Clinical and laboratory features of 300 patients and characterization of an intermediate group.

A series of 55 patients with B-cell chronic lymphocytic leukaemia (CLL) and prolymphocytic leukaemia (PLL) was studied in order to investigate the evolution of prolymphocytoid transformation of CLL. Peripheral blood films were assessed for the percentages of prolymphocytes (%PROL) during a followup period of 2 months to 24 years. The majority of patients with typical CLL (less than or equal to 10% PROL) had minor variations or transient increases in %PROL throughout the course of the disease, but in one third of them a steady rise in the proportion of these cells was documented. Patients presenting with 11-55% PROL, referred to as CLL/PL, exhibited three patterns of evolution: half of them showed stability of the PROL counts, one third had unsustained increases in the %PROL and 18% showed definite progression to a PLL-like blood picture. Patients diagnosed as PLL (greater than 55% PROL) had maintained a high %PROL during the period of observation. Serial marker studies in some CLL and CLL/PL cases showed that the percentage of M-rosettes and the intensity of SmIg remained at the same initial levels in all but two cases. These two cases in the CLL/PL group showed a significant decrease in the percentage of M-rosettes and stronger SmIg staining associated with progressive prolymphocytoid transformation. Patients with CLL and CLL/PL who had a sustained rise in the %PROL responded poorly to treatment compared with those with stable disease.

The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. II. Patterns of evolution of 'prolymphocytoid' transformation.

Chronic lymphocytic leukemia is a hematologic neoplasm characterized by proliferation and accumulation of mature-appearing lymphocytes. Most cases involve a clonal proliferation of B lymphocytes. The cells typically have low levels of surface immunoglobulin; usually mu or mu and delta heavy chains, and either kappa or lambda light chains. The cells also show receptors for mouse erythrocytes, Fc receptors for IgG, complement receptors, Ia antigens, and B-cell-associated antigens. Although chronic lymphocytic leukemia is usually a stable disease over months to years, transformation of both clinical and biological features may occur. Prognostic factors include the leukemia cell count (greater than 40 X 10(9)/L), anemia, thrombocytopenia, chromosome abnormalities, and the pattern of bone marrow involvement. Alkylating agents, radiation therapy, and corticosteroids are commonly used to treat patients with chronic lymphocytic leukemia. Although these agents are useful, few data show that survival has been substantially improved. Recently, biological response modifiers such as monoclonal antibodies and interferon have been studied.

Chronic Lymphocytic Leukemia: Recent Advances in Biology and Treatment

This report describes eight patients with B-cell chronic lymphocytic leukemia whose disease became more aggressive over a variable period of time. This clinical progression was associated with a change in cell morphology from small lymphocytes to an increasing number of large transformed lymphocytes in the blood, bone marrow, and lymph nodes. In the peripheral blood, the predominant large cell was a prolymphocyte. The small lymphocytes and the prolymphocytes had identical cell surface markers in each patient. However, the prolymphocytes had a greater density of surface immunoglobulin than did the same lymphocytes. No features were found that help predict in which patients CLL will convert to a more aggressive form. Once transformation has taken place, however, there appears to be a correlation between the number of prolymphocytes in the blood and patient survival. It is suggested that the entities of prolymphocytic transformation of CLL, prolymphocytic leukemia, and Richter's syndrome are less distinct than has been thought previously. These disorders probably represent several phases of transformation of the same cell type, and they may be examples of different stages in the natural history of CLL.

Prolymphocytic transformation of chronic lymphocytic leukemia.

T-cell lymphoma and the Chediak-Higashi syndrome.

Seven cases of multilobated lymphomas with B cell surface markers are presented Clinical, histological, ultrastructural and immunohistochemical features are detailed These observations serve to underscore the importance of not relying on morphology alone for the phenotypic classification of non-Hodgkin's lymphomas

Multilobated B cell lymphomas. A study of 7 cases.

Staining with peanut agglutinin (PNA) and with polyclonal antibody to S100 protein have both been recommended as aids in the diagnosis of histiocytosis X (Langerhans' cell histiocytosis). Although a large body of literature attests to the utility of S100 staining in this condition, the few published studies that involve PNA staining have employed varying methods of radically different results. We studied formaldehyde solution-fixed, paraffin-embedded tissue from 29 cases of histiocytosis X by using standard avidin-biotin immunostaining for S100 protein, as well as two published methods of PNA staining. All cases stained positively for S100 protein. By utilizing prior trypsinization and a three-step procedure, all cases also stained with PNA. With a two-step PNA-staining procedure, however, 9 cases failed to stain with PNA, and 3 cases showed a diffuse staining pattern that was not considered characteristic of Langerhans' and histiocytosis X cells. We concluded that both the S100 and the three-step PNA procedures are sensitive methods for the diagnosis of histiocytosis X.

A comparison study of two methods of peanut agglutinin staining with S100 immunostaining in 29 cases of histiocytosis X (Langerhans' cell histiocytosis)

J. Marty

Papers

Prolymphocytic transformation of chronic lymphocytic leukemia.

T-cell lymphoma and the Chediak-Higashi syndrome.

Multilobated B cell lymphomas. A study of 7 cases.

A comparison study of two methods of peanut agglutinin staining with S100 immunostaining in 29 cases of histiocytosis X (Langerhans' cell histiocytosis)

Health Risks in Preparation of the Esterase Stains