D. Lehoczky

Pregnancy in Liver Cirrhosis

Eleven patients with liver cirrhosis who had a pregnancy between 1974 and 1992 are reported. Prior to pregnancy 2 patients were splenectomized, 1 of them also had an unsuccessful mesocaval shunt and therefore underwent sclerotherapy. Furthermore, 3 patients were managed by injection sclerotherapy, 6 patients had episodes of hepatocellular failure and 5 had signs of hypersplenism. Gastrointestinal hemorrhage associated with pregnancy was noted in 6 patients. Jaundice was encountered in 2 patients, a raised bilirubin level in 3, ascites in 3, impairment of the synthetic liver function in 5, thrombocytopenia in 8, hemorrhagic diathesis in 5, and infectious puerperal complication in 5 patients. Esophageal sclerotherapy was used in 5 and transfusion in 6 patients. Of 12 births, 6 newborns were small-for-date and 1 of them died. Three neonates were preterm. Fetal wastage did not occur. The present data suggest that gastrointestinal hemorrhage in liver cirrhosis contributes to developing fetal growth retardation; cirrhotic patients can be prepared for pregnancy and the hematemesis during pregnancy can successfully be managed by esophageal sclerotherapy.

Pregnancy in idiopathic aplastic anemia (report of 10 patients)

This paper reports on 6 patients with severe, 2 with moderate and 2 with mild aplastic anemia who had a total of 18 pregnancies after the diagnosis. All four pregnancies that occurred during the active state of severe and moderate aplastic anemias were electively terminated. Two out of 14 pregnancies that occurred during the long-term remission were electively terminated for non-medical reason, two spontaneous abortions occurred and 10 live births were seen. All offspring were healthy at follow-up. During pregnancy the circulating blood cell levels decreased in 1 out of 6 pregnancies in patients who were in remission from mild and moderate aplastic anemias, and in 4 out of 8 pregnancies in patients who were in remission from severe aplastic anemia. In all 5 cases that showed a relapse during pregnancy the remission recurred following the termination of pregnancy. The data presented suggest that aplastic anemia in long-term remission can unpredictably relapse during pregnancy, but its final outcome appears not to be affected by pregnancy. Furthermore, there is no correlation between the pre-pregnancy clinical course and the events during pregnancy. The outcome of pregnancy during the remission of aplastic anemia seems beneficial, and spontaneous delivery should be preferred.

Hairy cell leukaemia: observations on natural killer activity in different clinical stages of the disease

Summary Follow‐up studies of natural killer (NK) cells, NK activity and antibody dependent cellular cytotoxicity (ADCC) in the course of hairy cell leukaemia (HCL) were carried out in a series of patients affected by the disease. NK activity against K562 targets was found to be high in all the patients with non‐symptomatic stable disease. On the other hand, absent or extremely low NK activity was found only in patients with symptomatic progressive disease. NK activity determined in the transitional stages showed values between these two extremes. Antibody dependent cellular cytotoxicity (ADCC) was less closely correlated with the clinical stage than NK activity. Our findings suggest that investigation of in vitro NK cell cytotoxicity might serve as a useful adjunct in determining clinical stage in HCL.

Immunological and molecular biological identification of a true case of T-hairy cell leukaemia.

A hairy cell leukaemia (HCL) patient is presented in whom the peripheral blood mononuclear cells (PBMCs) carried suppressor T‐cell markers (CD3 +, CD2 +, CD8 + /CD4‐, CD38 +). Analysis of genomic DNA of PBMNC showed the presence of a monoclonal population of T cells, the T‐cell receptor (TCR) beta‐chain gene being rearranged on both alleles (DR/DR), while the immunoglobulin (Ig) heavy chain‐genes were in germline configuration. The neoplastic cells were found to react with the monoclonal antibody RAB‐1 — originally described as belonging to the B lineage‐restricted monoclonal antibodies — and to carry RAB‐1/CD‐8 in a double marker assay. Natural killer activity of PBMNCs against K562 target cells was severely reduced, while the cells were found to exert strong antibody‐dependent cellular cytotoxicity.

OBSERVATIONS ON NK CELLS, K CELLS AND ON THEIR FUNCTION A LONG TIME AFTER POSTTRAUMATIC SPLENECTOMY

Natural killer (NK) cell activity and antibody-dependent cellular cytotoxicity of 48 posttraumatic splenectomy patients was found to be normal despite the increase in the proportion and the absolute number of large granular lymphocytes (LGLs). The NK cell activity of 2 further posttraumatic splenectomy cases was severely decreased, despite elevated number of LGLs and normal number of CD16+ peripheral blood mononuclear cells. Besides increased susceptibility to upper respiratory tract infections, 1 of these patients developed herpes genitalis recidivans. Two posttraumatic splenectomy patients were found to have developed chronic lymphocytic leukemia 5 and 31 years following splenectomy, respectively.

Development of chronic lymphocytic leukaemia after posttraumatic splenectomy

SummaryIn the course of a post-splenectomy follow-up study, 2 out of 102 patients who had been splenectomized after an abdominal trauma were found to have developed chronic lymphocytic leukaemia 5 and 31 years after splenectomy, respectively. The possible association between splenectomy and secondary leukaemia is discussed.

Responses to Single-Dose Thrombopoietin Decrease with Higher Platelet Counts in Mice

Since the description of human thrombopoietin (TPO) we investigated the thrombocytosis-inducing capacity of human serum samples derived from individuals with altered thrombocytopoiesis. Several times the degree of thrombocytosis developing in recipient mice differed markedly even when applying the same human material. In the last 2 years, we applied single doses of recombinant human TPO (rHuTPO) to random-bred CFLP mice, and the same observation was made. Taken together with previous information (before 1970) it was possible to select cases in which the percent increases in circulating platelet counts inversely correlated with the starting levels. It appears, however, that apart from the known absorbing role of platelets and megakaryocytes, the response to single doses of exogenous rHuTPO in mice depends, at least partially, on an unknown endogenous homeostatic mechanism. Mixing thrombopoietically active human sera with platelet-free normal serum in a 1:1 ratio remarkably reduced the thrombocytosis-inducing capacity. Repeated pharmacological doses of TPO, applied in the majority of the reported trials, however, easily obscure the physiological control mechanism.

Preferential localisation of red cell vacuoles to pathologically shaped human red blood cells.

The percentage of pitted erythrocytes and Howell‐Jolly bodies in peripheral blood samples of 51 individuals following posttraumatic splenectomy and 20 patients splenectomized because of various haematological diseases differed significantly from each other (p < 0.001) and from that of healthy controls (p < 0.001). The percentage of pitted erythrocytes was significantly higher in pathologically shaped red blood cells (RBCs) (acanthocytes, schizocytes, elliptocytes) than in normal discoid shaped RBCs (p < 0.001). As the number of pits per RBC showed great individual variations, a scoring system for the evaluation of pitted RBCs is proposed.