CLINICAL AND NEUROLOGICAL PECULIARITIES AND THE LEVEL OF AUTOANTIBODIES TO NEUROSPECIFIC ANTIGENS IN PATIENTS WITH CHRONIC HEMOBLASTOSIS

The purpose of this study was to determine the level of autoantibodies to neurospecific antigens and their relationship with clinical manifestations of the nervous system lesions in chronic hematological malignancies. Materials and methods. The study included 86 patients with chronic hemoblastosis divided into 3 groups: group 1 consisted of patients with chronic lymphoid leukaemia (n = 30), group 2 involved patients with chronic myeloid leukaemia (n = 30), group 3 included patients with multiple myeloma (n = 26). 21 inpatients of the neurological hospital without immunological and hematological disorders were selected as a control group. We investigated the anamnestic data on the presence of a clinical diagnosis of the nervous system lesions before and after the establishing a cancerous hematological diagnosis, carried out a complete neurological examination of the patients with chronic hematoblastosis to determine main neurological syndromes. The level of peripheral blood autoantibodies to neurospecific antigens was evaluated by the enzyme immunoassay. The level of circulating immune complexes was determined by the method of selective precipitation of antigen-antibody complexes. The findings of the study were statistically processes by using Microsoft Excel 2019 and IBM SPSS Statistics 26.0 software. The data obtained were processed by descriptive statistics methods. The study has demonstrated that encephalopathy (70%) and radiculopathy (40%) develop statistically significantly more often in the patients with chronic lymphoid leukaemia. The patients with chronic myeloid leukaemia are found to have encephalopathy (43%) and polyneuropathy (20%). The development of multiple myeloma is statistically significantly accompanied by the occurrence of radiculopathy (50%) and polyneuropathy (53%). Conclusion. The nervous system lesions are found considerably frequent in the patients with chronic hematological malignancies. The development of secondary encephalopathy in the patients with chronic lymphoid leukaemia may develop as a consequence of paraneoplastic syndrome due to the excessive production of cytokines and plasminogen activators. The predominance of radiculopathy in chronic myeloid leukaemia can be reliably explained by the high severity of the proliferative syndrome with a tendency to the production of localized chlorine, which can compress the corresponding roots of the peripheral nerves. However, peripheral lesions of nervous system are more typical for patients with multiple myeloma that may be due to hyperparaproteinemia combined with the chemotherapy of the underlying disease. Along with this, chronic lymphoid leukaemia and chronic myeloid leukaemia cause an increase in the circulating immune complexes level. Immunological changes indicate significant damage to nerve fibres and considerably rapid destruction of neurons in all forms of chronic hemoblastosis.

Cutaneous manifestation of leukaemia on the penis—the possible implications

Skin lesions are common in the patients with chronic lymphoid leukaemia (CLL); however, leukaemia cutis is a cutaneous manifestation secondary to any form of leukaemia and generally an uncommon phenomenon. They typically present on the face and neck as well as exposed areas. Our case looks at a 78-year-old gentleman with known CLL who presented with an asymptomatic raised lesion in his inner prepuce. The lesion was excised and his immunohistology staining confirms expression of CD5, CD20 and CD23 that is consistent with cutaneous manifestation of CLL. This case highlights the importance of taking leukaemia cutis into consideration in patients with known CLL with unusual features.

A másodlagos hypogammaglobulinaemia, a fertőzések és a halálozás összefüggései és a preventív immunglobulin-pótlás szükségessége krónikus lymphoid leukaemiás betegekben

Immune status was investigated in 186 patients with chronic lymphoid leukaemia between January 2012 and March 2015. Incidences of infections and mortality were analysed in patients who did not receive prophylactic immunoglobulin therapy. Immunoglobulin G (IgG) levels were normal (7–17.8 g/L) or decreased in 62.37% and 35.48% of patients, respectively. We measured high immunoglobulin levels only in a few cases (2.15%). Immunoglobulin levels became increasingly lower in more advanced disease stages (Rai stages). The number of infections was inversely proportional to that. Hypogammaglobulinaemia proved to be more important than disease progression in terms of the development of infections. The most common infections were upper respiratory tract (33.07%) and sepsis (18.90%). Two months after chemotherapy, initially normal immunoglobulin levels decreased by an average of 21%, and at the same time the incidence of infections increased. The most common cause of death was sepsis: 30% occurred at low immunoglobulin levels, while 20% at normal immunoglobulin levels. According to literature, prophylactic immunoglobulin treatment is indicated in patients with chronic lymphoid leukaemia and immunodeficiency for decreasing both morbidity and mortality. According to recommendations in literature, replacement treatment must be administered in severe or moderately severe recurrent bacterial infections. Immunoglobulin prophylaxis may be provided as low dose (10 g), fix dose (18 g) or individually customized higher dose (300–400 mg/kg body weight) treatment. According to recommendations, higher dose immunoglobulin prophylaxis, administered every three weeks on six occasions, is more efficient when customized. With this dose, infection-free condition may be achieved in 50% of patients. Orv Hetil. 2019; 160(38): 1487–1494.