[Unusual cause of macrohematuria in a 29-year-old woman]. / Seltene Ursache einer Makrohämaturie bei einer 29-jährigen Patientin.

HISTORY AND ADMISSION FINDINGS: We report on the case of a young women presenting with macrohaematuria, petechiae and strong headaches. INVESTIGATIONS: Laboratory showed a thrombotic microangiopathy with helmet cells, increased LDH levels (>600 U/l), and thrombocytopenia (<40,000/µl). DIAGNOSIS, TREATMENT AND COURSE: Due to strong haemolytic activity and headache with blurred vision, immediate plasma separation with fresh frozen plasma was commenced. Markedly decreased ADAMTS13 activity and detection of anti-ADAMTS13 antibodies were consistent with the diagnosis of idiopathic thrombotic thrombocytopenic purpura. In total, 11 plasma separations were required to stop disease activity. In parallel, immunosuppressive therapy using glucocorticoids was initiated. The patient was discharged from the hospital in a good general condition and with normalized laboratory findings 26 days after hospitalization. CONCLUSIONS: All patients with anemia and thrombocytopenia should be tested for haemolysis and helmet cells. An early diagnosis and initiation of necessary therapy are determining for the clinical outcome.

Anticardiolipin antibodies in serum and cerebrospinal fluid from patients with systemic lupus erythematosus.

Anticardiolipin antibodies were studied in serum and cerebrospinal fluid from 32 consecutive patients with systemic lupus erythematosus, admitted for the assessment of neuropsychiatric disease. Ten of the 16 patients with active neuropsychiatric complaints showed positive anticardiolipin antibodies in cerebrospinal fluid, including eight with the simultaneous presence of antibodies in their sera. By contrast, only 2 of the 16 patients with headaches, lacking further data of neurological disease, revealed anticardiolipin antibodies in their cerebrospinal fluid. The assessment of Q-albumin index showed abnormal values in a subset of patients with active neuropsychiatric changes who showed positive cerebrospinal anticardiolipin antibodies, suggesting that an impairment of the blood brain barrier function may lead to a leakage of intrathecal antiphospholipid antibodies from systemic circulation. Additionally, few patients revealed normal Q-albumin values with high IgG-cerebrospinal fluid index suggesting increased intrathecal synthesis of autoantibodies. The study of anticardiolipin antibodies in cerebrospinal fluid was useful to detect active neuropsychiatric disease in systemic lupus erythematosus.

[Immunologic and biochemical changes in patients with cranio-facial pain]. / Immunologicheskie i biokhimicheskie izmeneniia u bol'nykh kranio- fatsialgiiami.

The authors provide the results of an analysis of the interrelation between the immunologic and biochemical parameters in 6 groups of patients suffering from facial pains or headaches (a total of 153 patients). Significant correlations were revealed in the patients' groups with trigeminal neuralgia and periodic migrainous Horton's neuralgia. The main attention was concentrated on the following parameters: IgA in the serum, secretory IgA in the patients' saliva, % CD4 of lymphocytes and histamine concentration in the peripheral blood, concentration of beta-endorphin in the plasma, catecholamine content in the urine.

[Aspects of organ-specific autoimmunity in cluster headache]. / Aspetti autoimmunitari organo-specifici della cefalea a grappolo.

Fourty-eight cluster headache patients have been studied. Twenty-nine of them were affected also by rhythm cardiac abnormalities, and the other 19 by associated conjunctival hyperaemia. The presence of organ-specific autoantibodies was investigated by immunofluorescence. High titers of antibodies against cardiac antigenic determinants was found. The study of leukocyte subpopulations revealed a parallel increase in blood and in conjunctival mucosa of Leu7+ and LeuM3+ cells. These outcomes confirm the immunopathological theory of cluster headache.

Lymphokine-activated killer (LAK) cell phenomenon in cluster headache. "In vitro" activation by recombinant interleukin-2.

Previous studies showed that the Natural Killer (NK) activity of peripheral blood lymphocytes (PBL) from cluster headache (CH) patients is lower than that of controls. This decreased activity seems to be independent of the cluster period. beta-interferon has been shown to be more effective in increasing NK activity when incubated with PBL from CH patients, than with PBL from control donors. Lymphokine-Activated Killer (LAK) cells can be generated by incubation of human PBL in recombinant Interleukin-2 (rIL-2). This phenomenon was studied in 10 CH patients and 8 healthy volunteers. PBL were activated to LAK cells by "in vitro" incubation for 72 hours in Control Medium containing rIL-2 (1000 I.U./ml). A four hour Chromium 51 release was used to measure LAK Cell Killing of K562 target cells. The released radioactivity was measured in a gamma scintillation counter. The CH patients showed a marked increase of LAK generation compared to control subjects. This effect seems to be augmented during the cluster period.

[Local and general humoral immunity in patients with migraine, Horton's syndrome and autonomic pain]. / Opredelenie mestnogo i obshchego gumoral'nogo immuniteta u bol'nykh s migreniami, sindrom Khortona i vegetalgiiami.

Patients with migraines, Horton syndrome and autonomic pains were subjected to immunological investigation that revealed different degrees of local and general immunity disorders: increase in blood serum IfA and salival IgAc concentrations. These changes are believed to be capable of serving as diagnostic and prognostic indices.

[Immunoglobulins and the complement system in Horton's headache]. / Badania immunoglobulin i uklladu dopellniacza w bólu gllowy Hortona.

In 39 patients with Horton's headache (bgh) and 70 controls the total complement level and the levels of its C3 and C4 fractions, as well as IgG, IgA and IgM levels were determined. It was found that in bgh patients the mean level of the C3 fraction was significantly below that in the control group, while the C4 level was still within normal range. The levels of immunoglobulins were not statistically significantly different in these groups with the exception of IgA which was significantly lower in bgh cases. The author thinks that low C3 fraction level may be a part of the clinical pattern of bgh and is due to activation of the complement system on the alternative pathway. On the other hand, bgh attacks could not be qualified into any known type of allergic reaction.

[Levels of complement and its various fractions in the blood of patients with Horton's headache. Preliminary report]. / Poziom dopelniacza i niektórych jego frakcji we krwi chorych z bólami glowy Hortona. Doniesienie wstepne.

The pathogenesis of cluster headache remains unexplained and the immunological mechanisms have not been studied in this disease. Suspecting a possible allergic character of headache attacks it was decided to determine the haemolytic activity of the complement system and the levels of its C3 and C4 components. The investigations were carried out in 11 patients with cluster headache and in 10 patients with neurotic symptoms who served as controls. It was found that the levels of complement and its C4 fraction were not different in both groups but the level of C3 fraction showed a statistically significant difference. The authors discuss the obtained results and postulate continuation of investigations.