Albert I. Pick

Immunologic alterations in xeroderma pigmentosum patients

Nine xeroderma pigmentosum (XP) patients were investigated. In comparison to a normal control group the XP patients had a reduced OKT‐4 lymphocyte subpopulation, reduced response of lymphocytes to phytohemagglutinin in autologous serum, and diminished delayed hypersensitivity skin reaction. The possible contribution of ultraviolet irradiation to the observed immunologic alterations, and the link of these alterations to the susceptibility of patients for malignant transformation is discussed. Cancer 58:219–221, 1986.

Plasma cell myeloma associated with an unusual myeloma protein causing impairment of fibrin aggregation and platelet function in a patient with multiple malignancy

In a patient with threefold malignancy, chronologically hypernephroma, carcinoma of the colon and plasma cell myeloma, the latter was associated with an unusual M protein of Gm4 type, antigenically deficient when compared to reference proteins of the γ G1 heavy chain subclass. The patient suffered from epistaxis and had impaired fibrin clot formation and platelet function. The disturbance in fibrin clot formation was shown by spectrophotometry and electron microscopy to be due to interference by the M protein with fibrin aggregation. Equilibrium dialysis using 45 Ca did not demonstrate increased binding of calcium ions to the M protein. Ultracentrifugal study did not show association of the M protein to fibrinogen. In an advanced stage of the disease, plasma cell invasion into the peripheral blood occurred. Plasmapheresis, although partial, resulted in prolonged disappearance of the epistaxis and the plasmocytosis.

Monoclonal gammopathy in patients with chronic and acute myeloid leukemia

Monoclonal IgG components were found in the serum of 5 of 40 patients with chronic myelocytic leukemia (12.5%), as well as in 2 of 15 patients with acute myelocytic leukemia (13.3%). These findings may represent an involvement of the lymphoplasmacytic system in myeloproliferative disorders. The significance of this association is discussed.

Pleural effusion--presenting sign in multiple myeloma.

A patient with multiple myeloma in whom recurrent right pleural effusion was the presenting sign of the disease is reported. An IgA (k) monoclonal component was found in both the pleural effusion and the serum. The bone marrow specimen was interpreted as typical for multiple myeloma and the pleural fluid contained numerous plasma cells. Treatment with cyclophosphamide was followed by clinical improvement and the disappearance of the pleural effusion.

Relationship between immunoglobulin levels and extremes of solar activity

The possible relationship between epidemics and extremes of solar activity has been discussed previously. The purpose of the present study was to verify whether differences in the levels of immunoglobulins (IgA, IgG, IgM) could be noted at the highest (July 1989) and lowest (September 1986) points of the last (21st) and present (22nd) 11-year solar cycle. The work was divided into a 1-month study (covering the month of minimal or maximal solar activity), a 3-month study (1 month before and after the month of minimal or maximal solar activity) and a 5-month study (2 months before and after the month of minimal or maximal solar activity). A trend of a drop-off for all three immunoglobulins was seen on the far side of the maximal point of the solar cycle. Statistical significance was achieved in the 5-month study for IgM (P=0.04), and a strong trend was shown for IgG (P=0.07). Differences between the sexes were also noted.

Delayed hypersensitivity and lymphocytic transformation in patients with Hodgkin's disease and granulomas

Noncaseating, sarcoid‐like granulomas were found in the tissues of 9 out of 31 patients with Hodgkins disease. In vivo and in vitro cell‐mediated immunity was evaluated in patients with and without granulomas and compared to a group of 20 normal controls. Hodgkins disease patients of both groups showed a significantly reduced in vivo and in vitro response when compared to the control group. However, when patients in stages IIIB and IV were eliminated and patients in stages I, II and IIIA examined separately, a positive skin test response to one or more antigens was elicited in 85.7% of patients with granulomas, while a markedly decreased‐dose dependent response was observed in patients without granulomas. In vitro lymphocyte blastic transformation by phytohemagglutinin (PHA) was severely impaired in both groups of patients as determined by dose‐responses curves. These results indicate that Hodgkins disease patients with granulomas have a significantly better skin test response than those without granulomas.

Homogeneous populations of macrophages from histiocytic lymphoma patients as a source for macrophage subpopulations which differ in immunoregulatory properties

The existence of subpopulations of macrophages which express a variety of regulatory activities of other branches of the immune system is suggested in a comparative study of a human macrophage long term culture ZI and a macrophage cell line DAB‐1. Both cell cultures were derived from pleural effusions of patients with diffuse histiocytic lymphoma. DAB‐1 cells were found to secrete factors which strongly suppress the response of normal T and B lymphocytes to the mitogens PHA, Con‐A and pokeweed mitogen (PWM) (by 96–98%) and to stimulate the cytotoxic activity of NK cells.1 ZI cells secrete factors which have a mild inhibitory effect on the response of lymphocytes to the T‐cell mitogens PHA and Con‐A and a stimulatory effect on the response to PWM, whereas very little effect could be detected on the activity of NK cells. While DAB‐1 cells form large clusters during growth in culture, and are capable of inducing the formation of lymphocyte rosettes around the tumor cells, ZI cells grow as a homogeneous monolayer and could not be shown to form such rosettes. The differences in the behavior of the two cell populations suggest that the malignant transformation may have affected different subsets of macrophages in each case. Cells from histiocytic lymphoma patients may therefore be a source for homogeneous subpopulations of macrophages and their isolation and propagation in culture is one approach by which such subsets can be defined characterized and classified. The biological characterization of macrophage subsets may also be of clinical importance since a transformed subset with broad suppressory activities may lead to a violent and rapidly deteriorating course of disease, as was in the case of the patient from whom the DAB‐1 cell line was derived. Cancer 50:69–77, 1982.

Intestinal lymphoma with unusual sequence of serum IgA changes.

SummaryA patient with Mediterranean type of abdominal lymphoma presented an unusual sequence of serum IgA changes, initially elevated, then low, and finally excessive. The correlation between the IgA changes and the pathological findings in the small intestinal mucosa is discussed. Cervical lymphadenopathy of Hodgkins disease type developed in the course of the disease and is considered to have represented an additional malignant clone appearing consequent to immunological deficiency.

Acute Disseminated Intravascular Coagulation as the Presenting and Predominant Clinical Manifestation in a Patient with a Mucinous Adenocarcinoma

A patient with a fulminant clinical course of disseminated intravascular coagulation (DIC) is described. The DIC was first manifested by neurological symptoms and later dominated by hemorrhagic phenomena. Bleeding manifestations were not controlled by treatment with heparin, later supplemented with epsilon-amino-caproic acid and fresh blood. The patients condition precluded the performance of extensive diagnostic procedures. On the assumption that malignancy was the underlying disease, cyclophosphamide and hydrocortisone were administered. In spite of intensive treatment the patient died. Autopsy revealed mesenteric lymph node metastases of a mucinous adenocarcinoma, probably originating in the colon. It is emphasized that in some patients with malignant disease, acute DIC is the presenting and the predominant symptom. In such patients the hemorrhagic manifestations may not be controlled unless the underlying neoplastic disease is detected and treated.

Correlation between immunological parameters and cutaneous manifestations of systemic lupus erythematosus.

A patient with systemic lupus erythematosus with predominant skin involvement is described. A good correlation between disease activity manifested by skin lesions and between the titer of anti-DNA ant