A clinical case of pityriasis lichenoides chronica presenting with palpable purpura after streptococcal infection.

Pityriasis lichenoides is a rare inflammatory skin condition presenting with diffuse red-brown papules with evolution polymorphism and mica-like crust on older skin lesions. We present a 60-year-old female patient with pityriasis lichenoides chronica that manifested ten days after streptococcal pharyngitis. Initially, palpable purpura appeared on the lower extremities and later, erythematous-squamous papules and plaques appeared at the site of the palpable purpura and on the upper limbs and trunk. The patient had no history of hematological malignancy, viral hepatitis, kidney involvement, systemic rheumatic disease, or ANCA-associated vasculitis. After administration of methylprednisolone 20 mg for one month and an antimalarial agent (hydroxychloroquine 200 mg, 1 tablet bid) for three months, the skin lesions subsided without recurrence.

Durability of humoral and cell-mediated immune response after SARS-CoV-2 mRNA vaccine administration.

Vaccination against the SARS-Cov-2 virus is an effective way to protect against the disease and the severe course of COVID-19. Forty-nine fully vaccinated with mRNA vaccines (BNT162b2 or mRNA-1273) SARS-CoV-2 infection-naïve volunteers aged 33-89 were enrolled in the study. Evaluation of the cellular and humoral immune response was performed within 1 to 3 months (T1) and 6-9 months (T2) after the second injection, and within 2-3 months (T3) after a booster dose. Additionally, a comparative analysis of the specific immune status was made between two age groups-below 60 (n = 22) and over 60 (n = 27) years. SARS-CoV-2-specific T-cell response was evaluated by IFN-γ-producing spot forming cells (SFCs) using a standardized ELISPOT assay. Virus neutralizing antibodies (VNA) against SARS-CoV-2 were measured by a blocking ELISA test and spike protein specific IgG (S-IgG) and IgA (S-IgA) antibodies-by semiquantitative ELISA. IFN-γ-producing SFCs, S-IgG, S-IgA and VNA significantly decreased 6-9 months after the second dose. After the third injection S-IgG and S-IgA markedly increased compared to T2 and reached the levels at T1. Of note, the highest values of VNA were observed at T3. No differences in the tested immune parameters were found between the two age groups. Data obtained showed that for a long period-6-9 months after a full course of immunization with mRNA vaccine, immune reactivity is present, but both cellular and humoral immune responses gradually decrease. The administration of a third dose mainly restores the specific humoral immune response against the SARS-CoV-2 virus.

Biclonal myeloma in renal failure.

Multiple myeloma is a clonal proliferation of the plasma cell line that accounts for approximately 10% of all hematological malignancies. It is characterized by abnormal growth of plasma cells producing monoclonal immunoglobulin or light chain (paraprotein), with subsequent development of osteolytic bone lesions, anemia, hypercalcemia, and renal failure. In 3-6% of myeloma patients, more than one monoclonal protein (usually two) is discovered, with different heavy or light chain or both. These additional monoclonal proteins may be identified at the time of diagnosis or appear later during an observation or therapy. The authors describe two patients with biclonal myeloma, one diagnosed during evaluation for newly discovered renal failure, and one identified in the course of treatment of monoclonal gammopathy. The discussion of the diagnosis, natural history, and prognosis in patients with biclonal myeloma are also reported.

Common variable immune deficiency, central diabetes insipidus, and anemia.

Common variable immune deficiency (CVID) accounts for approximately 20% of all cases of primary immune deficiencies, and is characterized by low serum levels of IgG, IgA, and/or IgM. The diagnosis is usually made between 20 and 40 years of age, sometimes earlier. CVID patients are divided into two major groups based on complications observed: 1 group consists of patients with predominant infections, and 2 group includes patients with inflammatory and/or hematological complications, such as lymphadenopathy, splenomegaly, autoimmune cytopenia, enteropathy, and/or granulomatous conditions. The most prevalent gastrointestinal symptom is transitory or persistent diarrhea. Central diabetes insipidus (CDI) is a rare disease associated with decreased synthesis or release of antidiuretic hormone that leads to an excessive production of diluted urine (polyuria). Different factors can lead to the development of CDI, including autoantibodies to arginine vasopressin-producing cells. Celiac disease is an autoimmune condition affecting small intestine in genetically predisposed individuals, which can be associated with endocrinopathies. Here, we describe a patient with CVID, CDI, gluten-sensitive diarrhea, and anemia of combined type (thalassemia minor and B12-deficiency anemia).

A rare case of Addison's disease, hepatitis, thyreoiditis, positive IgG anti-tissue transglutaminase antibodies and partial IgA deficiency.

INTRODUCTION: Selective IgA deficiency (IgAD) is the most prevalent type of primary immune deficiencies, but partial IgA deficiency is even more common. Addison's disease is a rare condition associated with primary adrenal insufficiency due to infection or autoimmune destruction of the adrenals. The association between IgA deficiency and Addison's disease is very rare. CASE AND LABORATORY DATA: We observed a 22-year-old male patient with marked darkening of the skin, especially on the palms and areolae, jaundice on the skin and sclera, astheno-adynamia, hypotension (80/50 mm Hg), and pain in the right hypochondrium. The laboratory investigations revealed increased serum levels of total and indirect bilirubin, AST, ALT, GGT and LDH, negative HBsAg, anti-HBc IgM, anti-HCV and anti-HAV IgM, very low serum IgA levels (0.16 g/l) with normal IgG and IgM, negative ANA, ANCA, AMA, LKM-1, anti-GAD-60, anti-IA-2, anti-thyroglobulin antibodies, a mild increase in anti-TPO antibodies titer, a marked increase in IgG anti-tissue transglutaminase antibodies, with no typical changes in cellular immunity, negative T-SPOT-TB test, HLA - A*01; B*08; DRB1*03; DQB1*02, karyotype - 46, XY. CONCLUSIONS: We present a rare case of partial IgA deficiency with Addison's disease, hepatitis, thyroiditis and positive anti-tissue transglutaminase antibodies. IgAD and some autoimmune disorders share several predisposing HLA genes, thus explaining the increased prevalence of IgAD in certain patient groups.

Antiphospholipid antibodies in patients with upper-extremity deep vein thrombosis.

The levels of antibodies to cardiolipin and ß2-glycoprotein I and polymorphic variants G1691A of Factor V (factor V Leiden, FVL) and G20210A of prothrombin gene (G20210A) were studied in 16 patients with upper-extremity deep vein thrombosis (UEDVT). Most of patients with this syndrome have elevated values of these antibodies. Two of these patients are heterozygous carriers for G20210A and 1 - for FVL. Three patients with UEDVT and systemic lupus erythematosus (SLE) are positive for ANA and two others (one of them with Raynaud syndrome) have border line titre 1: 80 for ANA. All 3 patients with SLE are women and the interval between the development of the UEDVT and the onset of SLE was 1-4 years. We would like to suggest that: 1) UEDVT could be the first clinical symptom of Antiphospholipid syndrome, and 2) UEDVT may be the first clinical manifestation of SLE preceding the development of the systemic autoimmune disease by several years.

Bouquet variety of antiphospholipid antibodies in recurrent pregnancy loss.

Seventy-six female patients with two or more recurrent pregnancy losses (RPL) during the 1(st) trimester were studied. Based on the results of the aCL and aB2GPI antibodies testing, patients were divided in two groups: 22 patients with RPL and elevated immunoglobulin (Ig) G/IgM aCL and/or aB2GPI [RPL + antiphospholipid syndrome (APS)] and 54 patients with RPL alone (without high antibodies). Immunoglobulin G aPS and IgG a-AnV in patients with RPL + APS were higher than in controls and IgG aPS were higher in RPL + APS than in RPL alone. Additionally IgG a-AnV and IgM aPE are higher in RPL alone than in controls. In 18/22 (81%) patients with RPL + APS and 29/54 (54%) patients with RPL alone, there were one or more positive antibodies: aPS, aPT, a-AnV or aPE. These results raise a question whether or not these antiphospholipid antibodies should be routinely tested in women with RPL and especially in the context of the so-called "seronegative APS".