תוצאת חיפוש

(2,1) בולסלב קנובל, (1) אנה מיכלין,

(1) מח' לרפואה פנימית ב', מרכז רפואי וולפסון, חולון, (2) הפקולטה לרפואה סאקלר, אוניברסיטת ת"א

אשה בת 80 הלוקה בהיפותרמיה קשה (27 מ"צ), בסוכרת מסוג 2 (מטופלת באינסולין) ובחמצת מטאבולית ולקטית, נמצאה שוכבת על הריצפה בביתה עקב נפילה. היא שקעה בתרדמת עמוקה עם מחסור המודינאמי קיצוני, והרופאים המטפלים נטו לקבוע את מותה. בהתקבלותה לבית החולים נזקקה להחייאה, להנשמה ולחימום. ניצפו הפרעות קשות בחילוף החומרים, חמצת מטאבולית עם פער אניונים (anion gap) גבוה. הודגם תרשים אק"ג אופייני להיפותרמיה. כמו-כן לקתה הקשישה בראבדומיוליזיס (rhabdomyyolysis), באי-ספיקת כליות ובפגיעה בתיפקודי כבד. למרות הסיכוי הקלוש, החלימה החולה לחלוטין ושוחררה לביתה במצב בריאותי תקין.

Malignant Humoral Hypercalcemia Associated with Angiotropic Large B Cell Lymphoma: Case Report

Boleslaw Knobel, MD;^1,5 Ilan Sommer, MD;¹ Pauline Petchenko, MD;² Dorit Lev, MD; ³ Elimelech Okon, MD;^4,5

¹Department of Medicine "B", ²Department of Hematology, ³Institute of Genetics, Edith Wolfson Medical Center, Holon ⁴Department of Pathology, Rabin Medical Center, Petach Tikva and 5Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

Angiotropic large B cell lymphoma (angiotropic LCL) or intravascular large cell lymphoma (IVLCL) was diagnosed by liver and bone marrow biopsies and immunohistochemical studies in a 52 year old Caucasian male.

IVLCL is a very rare disease characterized by widespread intravascular proliferation of lymphoma cells. Although it most commonly affects the central nervous system or skin and occasionally bone marrow, angiotropic LCL may be present without evidence of localized disease, as seen initially in our patient.

To date, only a few cases of intravascular malignant lymphomatosis associated with parathyroid hormone related protein (PTH-rP) induced humoral hypercalcemia have been published.

Our extraordinary case was diagnosed mainly by liver biopsy. The neoplastic lymphoid cells stained diffusely and strongly positive with CD-20 (Pan B) and were negative for CD-3 (Pan T) immunostain.

The most significant, initial clinical finding was severe, unexplained hypercalcemia (until 18.6 mg/dl). Plasma PTH-rP showed a ten-fold increase at 8 pmol/L (normal value less than 0.8 pmol/L). Very unusual cytogenic abnormalities were found.

The patient received the massive third generation combination chemotherapy comprising of Methotrexate, Doxorubicine, Cyclophosphamide, Vincristine, Prednisone and Bleomycin and developed, complete although temporary, clinical, humoral and cytogenetic remission.

Cholesterol Pericarditis Associated with Rheumatoid Arthritis

B. Knobel, P. Rosman

Dept. of Medicine B, Edith Wolfson Medical Center, Holon; and Sackler Faculty of Medicine, Tel Aviv University

Cholesterol pericarditis (CP) is a rare and unusual disease characterized by chronic pericardial effusion with high cholesterol concentration. Precipitation of cholesterol crystals may occur and induce inflammation and constrictive pericarditis. CP may be idiopathic, but is usually associated with a systemic disease, such as tuberculosis, myxedema, or as in our case, rheumatoid arthritis (RA).

We present a 78-year-old woman with RA, typical deformities of the metacarpo- and metatarso-phalangeal joints and subcutaneous rheumatoid nodules. She was hospitalized with increasing dyspnea and weakness and a 2-dimensional transthoracic echocardiogram showed a large pericardial effusion, without tamponade.

Blood cholesterol was 208 mg/dl, triglycerides 169 mg/dl, LDH 37 u/L and rheumatoid factor 2560 u; glucose, kidney, and thyroid function tests were normal and PPD test negative. Pericardiocentesis yielded 800 ml of opaque, cloudy fluid, with glucose 19 mg/dl, cholesterol 264 mg/dl (normal 20-40 mg/dl), triglycerides 169 mg/dl, LDH 5820 u/L and rheumatoid factor 40 u; viral titers and cultures for bacterial, mycobacterial and fungal infections were negative. The pericardial fluid had a distinctive scintillating, gold-paint appearance and many cholesterol crystals were evident microscopically.

The patient responded to treatment with methotrexate and steroids. Factors responsible for increase in pericardial fluid cholesterol may be its liberation from injured pericardial cells and rheumatoid nodules, lysis of red cells, or lymphatic obstruction and impairment of the absorptive capacity of the pericardium.

Follicular Splenic Lymphoid Hyperplasia Associated with EBV Infection

Boleslaw Knobel, Enrique Melamud, Sharon Nofech-Moses, Liliana Zeidel

Dept. of Medicine B and Institute of Pathology, Edith Wolfson Medical Center, Holon and Sackler Faculty of Medicine, Tel Aviv University

Massive splenomegaly is defined as a spleen weighing about 10 times normal weight. We describe a 36-year-old man who had huge splenomegaly and secondary pancytopenia simulating malignant lymphoma for about 3 months. Splenectomy was necessary because of the suspicion of hematologic malignancy, especially isolated lymphoma of the spleen, and pain and mechanical abdominal disturbances.

On operation, the spleen was 25 cm long and weighed 250 g. There was florid, reactive follicular lymphoid hyperplasia. Immunohistochemical staining with CD-20(L26), CD-45Ro(UCHL), bcl-2 oncoprotein (Dakopatts), EBV (anti-EBV mol weight 60 KD, Dakopatts) was consistent with reaction to EBV infection and not with follicular lymphoma. Lack of PCR amplification using DNA extracted from paraffin-embedded splenic tissue indicated absence of a monoclonal B cell population carrying rearranged immunoglobulin genes. The lymphocytic population was proven polyclonal by the negative results of PCR for the bcl-2 gene rearrangement. EBV seroconversion from high titer antibodies of anti-EBV-VCA-IgM to negative, and from negative EBNA to positive was consistent with an apparent primary EBV infection.

We have not found on computerized search a previous report of reactive follicular splenic hyperplasia to EBV infection causing huge splenomegaly, with or without EBV-induced infectious mononucleosis.