Aggressive natural killer-cell leukemia with jaundice and spontaneous splenic rupture: a case report and review of the literature.

Aggressive natural killer cell leukemia/lymphoma (ANKL) is a rare aggressive form of NK-cell neoplasm. We report an uncommon case of 36-year-old male who showed jaundice and spontaneous splenic rupture. The diagnosis was established by the biopsy of liver and spleen. The monomorphous medium-size neoplastic cells infiltrated into portal areas and sinus of liver as well as the cords and sinus of the spleen. Necrosis, mitotic figures and significant apoptosis could be seen easily. These neoplastic cells demonstrated a typical immunophenotype of CD3ε+, CD56+, CD16+, Granzyme B+, TIA-1+. T-cell receptor γ (TCR-γ) gene rearrangement analysis showed germline configuration and the result of in situ hybridization for Epstein-Barr virus-encoded RNA (EBER-ISH) was positive. The patient has undergone an aggressive clinical course and died of multi-organ function failure 14 days later after admission. To the best of our knowledge, this is the first case of ANKL with spontaneous splenic rupture, and we should pay more attention to recognize it. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2048154883890867.

Effect of non-operative management (NOM) of splenic rupture versus splenectomy on the distribution of peripheral blood lymphocyte populations and cytokine production by T cells.

Post-traumatic splenectomy is associated with increased postoperative morbidity and mortality and long-term impairment of humoral and cellular immunity. Alternatives to surgery have been developed to minimize or avoid the immediate and/or long-term complications of splenectomy. Herein we investigated the long-term effect of non-operative management (NOM) of the traumatic rupture of the spleen on the distribution of peripheral blood (PB) lymphocyte populations and cytokine production by T cells. PB samples were drawn from six NOM patients, 13 age-matched adults who had undergone splenectomy after trauma (SP patients) and 31 age-matched controls. Cellular phenotypes and the intracellular production of interferon (IFN)-gamma, interleukin (IL)-2, IL-4 and IL-10 cytokines in T cells were determined in whole blood +/- mitogens by flow cytometry. NOM patients did not show any changes in the absolute numbers of lymphocytes or the distribution of their subsets, compared to the controls. In contrast, SP patients showed a sustained increase in the percentage and/or absolute numbers of lymphocytes, CD8 T cells, activated CD8 T cells, natural killer (NK) T cells, NK cells and gammadelta T cells, and a reduction in naive CD4 T cells. The constitutive or induced cytokine production by T cells of the NOM group was similar to the control group, whereas SP patients had increased percentages of constitutive IL-2- and IFN-gamma-producing CD8 T cells and IFN-gamma-producing CD4 T cells. Our findings indicate collectively that the healing process in NOM does not affect the architecture of the spleen to such an extent that it would lead to long-term alterations of the proportions of PB lymphocytes or the T cell cytokine profiles.

[Functional asplenia after severe varicella zoster-infection diagnosis by colour Doppler ultrasound]. / Autosplenektomie nach Varizellensepsis bei einem Patienten mit M. Crohn unter immunsuppressiver Therapie.

Infiltrative, inflammatory or thromboembolic processes in the parenchyma of the spleen can cause a functional loss of the organ. This phenomenon is called functional asplenia and occurs as a complication especially in sickle cell disease, lupus erythematosus and after bone marrow transplantation. We present the case of a patient with Crohn's disease under immunosuppressive therapy who developed a spontaneous covered spleen rupture in the course of a septic shock with DIG due to a Varizella zoster infection. Later on, sonography showed a diminution of the spleen size. No flow signals could be derived by colour doppler measurements from the spleen. Because of the colour doppler findings we suspected a functional asplenia which was then verified by spleen scintigraphy and Howell-Jolly-Bodies in the blood count. Remarkably, the Crohn's disease remains in complete remission since the development of the functional asplenia (for 4 years now). The underlying pathomechanism remains unclear.

A safe surgical technique for the partial resection of the ruptured spleen. A clinical report.

A consecutive series of 11 patients with an acute blunt splenic injury were treated with a 'safe resection' technique. 57% of the injured spleens (range 35-100%) were saved. None of the patients had any signs of secondary bleeding in control CT scan and the mortality was zero. No second-look laparotomies were performed. Follow-up time was at least two months (range 2 month-6 yr). Operation time was in average 120 min. Total mean peroperative bleeding was 1400 ml. Partial resection may offer patient a change for normal function of the injured spleen. However, it is not yet known what is the critical mass of spleen tissue needed for humans. The follow-up time of the present study is still too short to estimate this fact, but further studies may show the benefit of the present method in avoiding serious long term immunological complications of splenectomy. This present study introduces a novel technique for partial resection of injured spleen. Operation can be performed safely and quickly with a complication risk comparable to splenectomy. Resection is applicable even for multi-trauma patients.

Ruptured spleens with expanded marginal zones do not reveal occult B-cell clones.

An indolent variant of splenic marginal zone lymphoma (SMZL) lacking massive splenomegaly has been described as an incidental finding in spleens removed for rupture or hypersplenism. We studied traumatically ruptured spleens with expanded marginal zones (MZs) to assess the incidence of occult monoclonal B-cell populations in this setting. Ninety-one ruptured or lacerated spleens removed from 1984 to 1995 were classified as to whether they had expanded MZs (> 12 cell layers thick). When available, paraffin-embedded, formalin-fixed tissue from cases with expanded MZs was examined for immunoglobulin heavy chain gene rearrangement by polymerase chain reaction (PCR) and stained for CD20, CD43, and kappa and lambda light chains. Splenectomies were performed for blunt (70 patients) and penetrating (7 patients) trauma, surgical misadventure (13 patients), or spontaneous rupture (1 patient). There were 58 men and 33 women in our study, ranging in age from 17 to 87 years (mean, 40 yr). Average spleen weight was 183 g (range, 44-505 g). Twenty-seven (30%) of 91 patients had expanded MZs. There were no significant differences in age, sex, spleen weight, or reason for excision between those cases with and without MZ expansion. Germinal centers varied from absent to inactive to floridly reactive. Paraffin blocks were available in 24 cases; the 20 with amplifiable DNA were polyclonal by PCR. Follow-up was available for 25 of the 27 patients with expanded MZs (range, 1-85 mo; median, 6 mo); lymphoma did not develop in anyone, although one patient's spleen was morphologically suspicious for lymphoma, showing involvement of red pulp by MZ-type B-cells; PCR revealed a polyclonal pattern. This patient's 3-year follow-up revealed no evidence of lymphoma. Traumatically ruptured spleens with expanded MZs do not seem to harbor occult B-cell clones, as detected by PCR. Although a few cases of incidentally removed spleens have been reported to contain low-stage SMZL, this seems to be an infrequent event.

The ruptured spleen. A histological, morphometrical and immunohistochemical study.

A traumatically ruptured spleen is regarded as a proper control in many histological and immunological studies on the human spleen. This paper compares spleens that ruptured due to trauma and spleens which were removed during surgery in patients without splenic pathology. Based on a histological, morphometrical, and immunohistochemical description of the control spleens it is shown that the traumatically ruptured spleens contain alterations in the lymphoid tissue. The amount of white pulp is increased due to a larger amount of CD4-positive lymphocytes. Furthermore there are alterations in lymphocyte populations in the different splenic compartments. It is concluded that spleens that rupture may be predisposed due to immunological stimulation.

Antibody response to pneumococcal vaccine in splenectomized adults and adolescents.

The antibody response of 67 splenectomized adults and adolescents with benign underlying diseases to a 14-valent pneumococcal capsular polysaccharide vaccine was determined by an enzyme-linked immunosorbent assay. It was not significantly different from that of 12 healthy non-splenectomized adult volunteers for 13 of the 14 polysaccharide antigens studied. Residual splenic tissue as detected by Tc-scintigraphy was without any influence on the vaccination response. In comparison 5 untreated splenectomized adults with malignant diseases and 11 splenectomized adults receiving immunosuppressive therapy exhibited a significantly reduced combined geometric mean of their postvaccination antibody concentrations (all 14 antigens added) and of their combined geometric mean antibody fold increase as compared to the healthy non-splenectomized adults. The reduction in antibody response was most pronounced in the group of immunosuppressed patients. Immunogenicity of each of the 14 vaccine antigens varied considerably as judged by the geometric means of the postvaccination arbitrary antibody concentrations. Also individual variation in postvaccination antibody concentration against each antigen was large.

Suppressor T cell activity in splenectomized subjects.

The effect of splenectomy on the expression of suppressor activity of peripheral blood lymphocytes was examined in this study. 63 soldiers splenectomized for trauma or hematological diseases one to 24 years earlier, were studied by measuring the suppressor cell activity (using the Con A method), and lymphocyte blast transformation in response to lectines (PHA, Con A, PWM). There was a decrease in suppressor cell activity in 15 (23.8%) of the splenectomized patients. This decrease was greater in subjects splenectomized for trauma than in those splenectomized electively (24% and 22% respectively). The decrease was related to length to time from surgery being highest in the first 5 years after removal of the spleen and was more frequent in females (43%). The mean of the stimulation indices calculated from the mitogenic response to PHA, PWM and Con A was lower in the splenectomized patients with reduced suppressor activity than in those with normal suppressor activity. The findings of our present study supports the view that splenectomy interferes with normal immunoregulatory mechanisms and suggests that pyogenic infections are not the sole hazard of this procedure.

Serum immunoglobulin levels in splenectomized Hodgkin patients and in subjects following post-traumatic splenectomy.

Mean serum immunoglobulin levels (IgG, IgA, IgM) in splenectomized Hodgkin's disease patients in remission were compared with a control group of splenectomized healthy subjects following trauma. The controls showed a higher IgA and lower IgM than the normal. The IgM of the Hodgkin's group were decreased below the normal but showed no difference from the control group. IgA and IgG levels were not significantly different from the levels in the splenectomized healthy subjects. These results suggest that changes in immunoglobulins in splenectomized Hodgkin patients could be attributed to the effect of the removal of the spleen.

Spontaneous rupture of the liver and the spleen: report of a successfully treated case with immunopathologic reaction.

A patient with a spontaneous rupture of the liver, followed by a spontaneous rupture of the spleen and gastrointestinal bleeding, is reported. The patient was operated for both ruptures and survived. We could not find any corresponding reports in the literature. Histological findings both in the liver and in the spleen were suggestive of an acute immunologic reaction and the symptoms of the patient resembled those of an autoimmune disease. Attention is drawn to the possibility that the ruptures of the liver and the spleen might have represented an allergic reaction reminiscent of the general type of Schwartzman reaction. This also could explain the other systemic manifestations of the disease. In the present patient steroid therapy eliminated all symptoms, and this possibility should be kept in mind when treating obscure ruptures of the liver and the spleen.