摘要:

SummaryThe umbilical cord blood from 109 consecutive Zambian neonates (excluding those found to be anti‐HIV positive) were analysed for haemoglobin (Hb) Bart's and for alpha thalassaemia by restriction endonuclease analysis. This showed that 52.3% had the genotype αα/αα, 38.5% had ‐α3.7/αα, 7.3% had—α3.7/—α3.7and 1.8% had ααα/ααα. The alpha thalassaemia gene (—α) frequency was 0.27. There were no apparent differences in gene frequency between six major Zambian ethnic groups. Detection of Hb Bart's identified all α‐thalassaemia homozygotes (—α/—α), but fewer than 10% of heterozygotes (—α/ααα), α‐thalassaemia was associated with slig

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00167.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummaryClinical palpation of the spleen was assessed by reference to a radionuclide scan method for measuring spleen volume in vivo fromV= 9.88/4 — 534, whereV= volume (cm3) and A — posterior scan area (cm2) (Zhang&Lewis 1987). The position of the spleen tip in relation to the costal margin correlated well with clinical palpability when the spleen was clearly enlarged, but when the spleen tip was just below the costal margin it was not always palpable. In some cases a normal‐sized spleen was palpable, whereas a non‐palpable spleen was not always normal sized. As the scanning method gives an accurate measurement of spleen size it is especially useful in lesser degrees of enlargement when reliable information on the splenomegaly may help to elucidate a diagnosis and problems of man

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00168.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummarySevere haemorrhagic thrombocytopenia in infectious mononucleosis with positive Epstein–Barr virus (EBV) serology remains a threatening clinical problem. Although rare, fatalities have been reported. We present clinical, haematological and serological details of two patients with diffuse bleeding associated with this disease who failed conventional steroid treatment and who both responded promptly and efFectively to infusions of intravenous immunoglobulin (i.v. Ig). A platelet immunofluorescence test was used to detect platelet antibodies in acute and recovery phase patient sera against autologous recovery platelets and against normal allogeneic group O platelets. In one patient, indirect immunofluorescence using recovery platelets detected an IgG platelet autoantibody with acute phase autologous serum. No platelet antibody was detected in the second patient. The data presented demonstrate that response to i.v. Ig is independenl of serologica) detection of platelet autoantibodies. Therapy with i.v. Ig should be considered in any case of haemorrhagic thrombocytopenia associated with EBV infection shown to be refractory to steroid treatmen

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00169.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummaryEarlier studies have indicated a role for serum lactic dehydrogenase (LDH) in haematological and other malignancies. As this is still not a routine test in many units, its value has been re‐examined in a retrospective analysis of serum LDH levels in 278 patients with haematological malignancies managed in a single unit. Abnormal levels at diagnosis were found in 42% of these disorders; yet analysis of over 23 000 routine requests for a biochemical screen showed only 4% with an abnormal LDH level. The estimation was also valuable in follow‐up of those haematological patients, with rising levels often indicating progressive or relapsing disease even in cases which had shown a normal LDH at diagno

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00170.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummaryBlood samples from 21 patients with confirmed hereditary spherocytosis (HS) were studied using the Technicon H1 blood counter. Nine patients had had a splenectomy and 12 were unsplenectomized. In the latter, the means of MCHC and RDW as measured by the H1 were significantly higher than results obtained using the Technicon H6000. Furthermore, spherocytes were recognizable in all of the H1 histograms of red cell size and Hb concentration as distinctive hyperchromic and microcytic tails, and the HDW (Hb distribution width) was consistently elevated. In the splenectomized patients, although the mean MCHC and RDW results were significantly higher as measured by the H1, it was not possible to recognize any abnormalities in the red cell histograms, and the HDW results were normal. Two of 75 apparently normal blood donors showed very small tails of hyperchromic cells, but in neither was this sufficient to disturb the MCHC, RDW or HDW parameters, and confusion with an unsplenectomized case of HS would be very unlikely. The H1 appearances of HS are so specific that the measurement of osmotic fragility will seldom be necessary.

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00171.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummaryPeripheral blood lymphocytes from 20 patients with neutropenia not consistent with aplastic anaemia were tested for their ability to inhibit the proliferation of normal granulopoietic precursor cells (CFU–GM) in agar culture. Two patients, both with features of an autoimmune disorder, had lymphocytes which were more inhibitory than normal lymphocytes to both normal and their own CFU–GM. Two other patients had lymphaocytes which were more inhibitory than normal lymphocytes to either their own CFU‐GM or normal CFU–GM but not both. Eight patients had lymphocytes which were significantly less inhibitory than normal lymphocytes to either normal or their own CFU‐GM, but only one showed this feature against both normal and their own CFU‐GM. One patient had a highly potent plasma inhibitor of CFU‐GM — this patient had received multiple transfusions and had a leucocyte antibody of a broad specificity. No clinical or haematological features were common to any of these groups of patients which reflects the heterogeneity of patients studied and stresses the importa

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00172.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

SummaryThe response of human monocytes to red cells sensitized with known levels of monoclonal antibody to the Rh antigen D (anti‐D) was compared with that of polyclonal anti‐D. Monocyte response was determined by measuring red cell adherence, erythrophagocytosis, monocyte‐mediated red cell lysis and luminol‐dependent chemiluminescence. By all criteria, monoclonal and polyclonal antibodies showed comparable activity, with IgG3 antibodies promoting a greater monocyte‐red cell interaction than IgG1 antibodies. It is suggested that monoclonal anti‐D may be effective in the prophylaxis of haemolytic disease of the newborn, providing such material is clinically

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00173.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Pyrimidine 5′‐nucleotidase (5′‐ribonucleotide phosphohydrolase, E.C. 3.1.3.5) (P5N) deficiency is one of the more common causes of hereditary non‐spherocytic haemolytic anaemia. This syndrome is characterized by haemolytic anaemia, pronounced basophilic stippling of red cells, and marked increase in red cell pyrimidine‐containing nucleotides (Valentineet al.1974). P5N activity is quantitatively measured by a colorimetric method (Valentineet al.1974) and a radiometric method (Torrance, West&Beutler 1977). For screening of P5N deficiency, the recommended method is to determine the u.v. absorption of blood extract (Be

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00174.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

The association of chronic lymphocytic leukaemia (CLL) and other tumours has been frequently described. Involvement of the breast by CLL is rare. Only four cases of this association have been described (Haram 1937; Sealeet al.1972; Desablenset al.1985). We report here a case of B‐cell CLL presenting with a lump in the breast which may indicate that the disease has a more aggressive clinical outloo

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00175.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

Acquired pure red cell aplasia (PRCA) in adults is an uncommon condition which is associated with a thymoma in approximately 50% of patients (Ammus&Yunis 1987). It has also been reported in association with a wide variety of other conditions including occasional haematological malignancies when it occurs at the height of the disease, and carcinoma when the red cell aplasia may precede the diagnosis of malignancy by up to 3 years (Ammus&Yunis 1987).We report two cases of PRCA which antedated the appearance of chronic myelomonocytic leukaemia (CMML) by a period of 3 months.

ISSN:0141-9854    

DOI:10.1111/j.1365-2257.1989.tb00176.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY