Spontaneous splenic rupture during induction chemotherapy for acute promyelocytic leukemia successfully treated with splenectomy

We report a case of acute promyelocytic leukemia who suffered spontaneous splenic rupture with massive hemoperitoneum while receiving intensive induction chemotherapy. Emergency computed tomography of the abdomen helped in the diagnosis of intra-abdominal bleeding. The patient was successfully treated with immediate splenectomy and made an uneventful postoperative recovery. Ten days after surgery chemotherapy could be resumed and complete remission was achieved. Although spontaneous splenic rupture is a rare complication of hematologic malignancies, this diagnosis should be considered in all patients with leukemia who develop acute abdominal pain with hypotension, even in the absence of splenomegaly.     

Pathological splenic rupture: a rare complication of chronic myelomonocytic leukemia

Chronic myelomonocytic leukemia (CMML) is an uncommon disorder characterized by monocytosis of the peripheral blood, absence of the Philadelphia chromosome, fewer than 20% blasts, and one or more lineages showing dysplastic features. Splenomegaly is frequently seen and may be massive. A 56-year-old man with stable CMML and moderate splenomegaly presented to the emergency department with generalized abdominal pain and abrupt drop in hemoglobin. Abdominal imaging revealed splenic rupture and emergency splenectomy was undertaken, with complete recovery. Atraumatic rupture of the spleen has rarely been reported as a complication of CMML or other myelodysplastic disorders. This report should alert physicians to consider this diagnosis in patients with CMML and acute abdominal pain.     

Life-threatening spontaneous splenic rupture with systemic lupus erythematosus: case report and literature review

Although involvement of the reticuloendothelial system in systemic lupus erythematosus (SLE) is a well-recognised concomitant of the disease, spontaneous splenic rupture is an unusual occurrence. We observed a 54-year-old woman with SLE who had spontaneous splenic rupture during the late course of the disease and showed some changes histopathologically. The courses and the clinical characteristics of such patients are reviewed, and the diagnosis and treatment of these cases are discussed. Early evaluation for SLE patients with spontaneous splenic rupture should be considered and included in the differential diagnosis of acute abdomen, as it may affect follow-up and treatment, although the condition is rare.     

Current approaches to the chemotherapy of B-cell chronic lymphocytic leukemia: a review

Standard therapy has not substantially improved the outcome of patients with chronic lymphocytic leukemia (CLL). However, an increased understanding of the biology and immunology of CLL, and the availability of several new and active chemotherapy agents (eg, fludarabine, 2'-deoxycoformycin [DCF], 2-chlorodeoxyadenosine [CDA]) has stimulated enthusiasm for clinical trials. DCF induces CRs or PRs in 25% of heavily treated patients. Fludarabine has been associated with a response rate of greater than 50% in previously treated patients, and greater than 70% in untreated patients, with almost a third of these achieving a CR. Currently, phase I and II clinical trials are evaluating combinations of these drugs with each other or with conventional agents (eg, fludarabine/chlorambucil [CLB]/prednisone [P]; DCF/CLB/P; fludarabine/DCF; fludarabine/P) in previously treated patients. To facilitate comparison of these regimens, each study is adhering to the NCl-Working Group guidelines for eligibility and response criteria, and toxicity assessment. A collaborative phase III trial will then compare the most promising of these regimens with "standard" chemotherapy in previously untreated patients. The widespread availability of these clinical trials will allow clinicians ready access to the new treatments.     

Portal hypertension caused by intra-hepatic block during chronic lymphoid leukemia

Portal hypertension in chronic lymphocytic leukemia is rare. A 66 year-old man was admitted for splenomegaly, thrombopenia and cholestasis. Endoscopy showed esophageal varices. The hepatic venous pressure gradient was 15 mmHg. The liver biopsy showed dense leukemia cells in sinusoidal and portal sites. After splenectomy, the hepatic venous pressure gradient normalized, but esophageal varices and cholestasis persisted. The authors discuss the mechanisms of portal hypertension in chronic lymphocytic leukemia. Previously reported cases are summarized.     

Relation of P-glycoprotein expression with spontaneous in vitro apoptosis in B-cell chronic lymphocytic leukemia

Prolonged lifespan of monoclonal lymphocytes in B-cell lymphocytic leukemia (B-CLL) arises from their resistance to programmed cell death. In contrast, when cultured in vitro, B-CLL tumour cells rapidly undergo apoptosis. There is mounting evidence that P-glycoprotein (P-gp), an adenosine triphosphate-binding cassette (ABC) family transporter, plays a significant role in the regulation of apoptosis induced by various stimuli. Since P-gp is commonly expressed in B-CLL cells, we aimed to establish whether its expression level influences resistance to spontaneous apoptosis in B-CLL. For that purpose, P-gp expression by UIC2 antibody staining and P-gp activity by rhodamine 123 (Rh123) efflux in presence or absence of P-gp inhibitor verapamil were studied in peripheral blood lymphocytes obtained from 43 previously untreated B-CLL patients. Simultaneously, the percentage of cells undergoing spontaneous in vitro apoptosis (apoptotic index, AI) by means of activation of caspases and annexin-V-based assays was evaluated. The AI were higher in B-CLL cells than in normal peripheral blood mononuclear cells (medians of AI 27.7% vs 3.9%, p=0.0001 and 34.7% vs 7.4%, p=0.0038, in 24 and 48-hour culture respectively). The AI were also higher among female patients as compared to male patients (medians: 29.7 vs 19.2 p=0.048). Interestingly, we found moderate inverse correlation between P-gp protein expression and AI after 24-hour culture in analysed B-CLL samples (r= -0.36, p=0.019). Moreover, P-gp positive B-CLL samples expressed significantly higher AI than P-gp negative samples with an arbitrary cut-off at Kolmogorov-Smirnov statistics D-value 0.2 (medians of AI 18.4% vs 29.7%, p=0.026). Based on these results we suggest that P-gp expression has some protective effect on B-CLL cell survival in vitro. The difference in the rates of spontaneous apoptosis among male and female patients may contribute to gender-dependent variations in clinical outcome in B-CLL.     

Acute abdomen caused by spontaneous rupture of splenic hydatid cyst

We report a rare case of acute abdomen caused by the spontaneous rupture of a splenic hydatid cyst into the abdominal cavity, causing a massive hemoperitoneum due to accompanying rupture of the spleen which required splenectomy. A review is made of the literature on this rare entity, its treatment and its consequences and their prevention and treatment.     

High frequency of monoallelic retinoblastoma gene deletion in B-cell chronic lymphoid leukemia shown by interphase cytogenetics

Inactivation of the retinoblastoma tumor-suppressor gene (RB-1) has been associated with tumorigenicity in various human malignancies. In chronic lymphoid leukemias of B-cell origin (B-CLL) an involvement of RB-1 has been suggested based on cytogenetic data. We examined RB-1 and its chromosomal locus 13q14 in 35 cases of B-CLL by dual-color in situ hybridization to interphase nuclei and by G-banding analysis of metaphase chromosomes. In one patient (pt) a monosomy 13, and in three other pts deletions involving or encompassing band 13q14 were detected by conventional cytogenetic analysis. In contrast, in situ hybridization to interphase nuclei showed a monoallelic RB-1 deletion in 11 cases (31%). One pt showed a translocation with the breakpoint in 13q1?4 on G-banding, but on in situ hybridization analysis the RB-1 signals were not affected. Our data show that RB-1 deletions can be diagnosed accurately by in situ hybridization on the one-cell level. The frequency of RB-1 deletions detected in this study is significantly higher than previously assumed in B-CLL, and seems to be in the same range as in retinoblastoma.