A case of the leopard (multiple lentigines) syndrome is described. To our knowledge this is the first documented case of this rare but interesting syndrome to be reported in this country.
A 72 year old man developed acute organic brain syndrome associated with marked eosinophilia following self medication with a variety of drugs. Investigations revealed no other known causes of eosinophilia. Withdrawal of drugs resulted in dramatic drop in eosinophil count paralleled by clinical resolution of neurological problems. To our knowledge drug-induced eosinophilia has not previously been associated with acute organic brain syndrome.
The simultaneous expression of both lymphoid and myeloid phenotypic features in acute leukaemia is rare. We report 3 cases of biphenotypic hybrid acute leukaemia seen in our institution. All 3 patients achieved remission with treatment for acute lymphoblastic leukaemia but two subsequently relapsed while on treatment. The hybrid acute leukaemias are important areas for further research both for delineation of basic biology and choice of optimal treatment.
A 47-year old man had hypersplenism from massive splenomegaly, the cause of which was undetermined for 2 years. He was initially asymptomatic though there was mild pancytopenia. However, 18 months after presentation he manifested both clinical and haematological deterioration, almost succumbing to sepsis. Splenectomy finally provided a definite diagnosis of follicular lymphoma and also restored his blood counts to within normal range.
Twenty patients with Myelodysplastic Syndrome (MDS) were diagnosed in University Hospital, Kuala Lumpur over a 5 year period. They were subclassified using the French American British (FAB) criteria. 90% of the patients were above 40 years old and the sex ratio was about equal. The predominant presenting symptom was anaemia and there was paucity of physical signs at presentation. Patients with 'aggressive' subtypes of MDS i.e. refractory anaemia with excess blasts (RAEB), refractory anaemia with excess blasts in transformation (RAEB(-)+) and chronic myelomonocytic leukaemia (CMML) had more frequent thrombocytopenia and neutropenia and their marrow pictures frequently had dysmegakaryopoiesis and dysgranulopoiesis as compared to more the "benign" subtypes i.e. refractory anaemia (RA) and refractory leukaemic anaemia with ringed sideroblasts (RARS). Four patients had leukaemic transformation and all of them came from the 'aggressive' subtypes. The current views on treatment of MDS are discussed.
A retrospective study of 37 cases of multiple myeloma admitted from 1980 to 1987 to the University Hospital Kuala Lumpur, Malaysia, was carried out to analyse the biodata, clinical presentation, laboratory and radiological profiles. The cases were selected after they had satisfied preset diagnostic criteria. The mean age was 60 years. There was no sex or ethnic preponderance. The most common symptom was bone pain. Pallor was detected in 73% of the patients. Haemoglobin was less than 120 g/L in 95%, and ESR was greater than 100 mm/hr in 70% of cases. Bone marrow and trephine biopsies were diagnostically important. Hypercalcaemia occurred in seven cases out of which three were IgA myelomas. Either serum creatinine or blood urea was raised in nearly 50% of cases. The most common heavy chain paraprotein was IgG while Kappa light chain was the commoner light chain type. 86% of cases had osteolytic lesions. These findings are, in general, similar to those of larger studies on multiple myeloma.
Study site: University Malaya Medical Centre (UMMC)
A young primigravida had idiopathic warm antibody (IgG) autoimmune haemolytic anaemia (AIHA) occurring in the third trimester of pregnancy. Her haemolytic process was responsive to steroid therapy and no transfusion was needed. She delivered a healthy baby with no evidence to haemolysis, though his red cells were coated with IgG which was probably of maternal origin.
A 27 year old housewife developed thrombotic thrombocytopenic purpura during the twelfth week of pregnancy. She had partial response to initial plasma infusion and subsequent plasmapheresis. However, her clinical course was complicated by the development of severe pancytopenia the consequence of a hypocellular marrow. She succumbed to septicaemic shock one month after diagnosis. The development of hypocellular marrow in thrombotic thrombocytopenic purpura has not been reported before.
In this retrospective study, 81 patients were diagnosed to have deep vein thrombosis (DVT) at the University Hospital, Kuala Lumpur over a ten year period from 1977 to 1986. Fifty-six patients had their DVT confirmed by venograms. The left side was affected in 79.5% of venographically proven cases of DVT of the lower limbs above the popliteal vein. The incidence of clinically apparent DVT following surgery was 0.21 per 1,000 operations. The incidence of pregnancy-related DVT was 0.039%. DVT was 3 times more frequent in the puerperium than in the antenatal period. The overall incidence of symptomatic pulmonary embolism was 13.6% of the cases of DVT.
Hereditary spherocytosis is a rather uncommon disease in Malaysia as only 16 patients were seen in our hospital over a 13 year period. Pallor, jaundice and splenomegaly were common physical signs. Clinical severity of the disease was variable and more than half of them needed splenectomy. Complications including haemolytic crisis and cholelithiasis were encountered but not aplastic crisis. All 10 patients who underwent splenectomy had uniformly good results and none of them had post-operative complications.
This paper reviews our first 100 consecutive PTCAs done between December 1988 and May 1990. There were 31 females and 69 males and their ages ranged from 37 years to 80 years. The mean age was 57.7 years. We achieved a follow-up rate of 66%. The restenosis rate was 22% of those that we could follow-up. There were 35 simple and 65 complex PTCAs. An average of 1.39 arteries were entered per PTCA. Of the 100 PTCAs done there was a primary success rate of 83%. On closer examination most of the failures were in the total occlusion group. There were 30 total occlusions done and 17 were successfully dilated. The primary success rate for acute total occlusions was 77% and the primary success rate for chronic total occlusions was 41%. Of the 100 PTCAs there were two deaths and only one patient had to go for emergency bypass surgery. The other complications were relatively minor including hypotension, groin bleeding, chills and rigors. This paper documents our initial 100 cases of PTCAs. It shows that our figures are compatible with other centres in this region and those around the world.
The case records of 64 patients with malaria over a five year period admitted to the University Hospital, Kuala Lumpur were examined. There were 32 cases of P. falciparum, 26 cases of P. vivax and two cases of mixed infections. Four cases of P. malariae were recorded. The clinical findings, biochemical and haematological parameters were examined for any indication of a pernicious syndrome. A high index of suspicion of a malarial infection may be based on the findings of anaemia, thrombocytopaenia, hyponatraemia, renal failure and abnormal liver function tests in the face of a negative blood film. These pernicious syndromes occur more often in malignant tertian malaria (anaemia 50%, hyponatraemia 39.1%) but a high percentage of the other malarial species show these abnormalities (P. vivax anaemia 57.7%, hyponatraemia 19.2%). When these abnormalities are present but blood films for malaria parasites are negative, repeat blood films are warranted until a parasitological diagnosis is achieved and correct treatment may be started.
A review of malaria cases over a five year period from 1984-1988 at the University Hospital, Kuala Lumpur, Malaysia is presented. A total of 64 cases were recorded; 50% of which were due to Plasmodium falciparum, 40.6% were due to Plasmodium vivax, 6.2% due to Plasmodium malariae and 3.1% due to a mixed infection of Plasmodium falciparum and Plasmodium vivax. The breakdown of species type compared similarly with other studies conducted in the region. Of this total, sixteen cases were imported from Pakistan, India, Thailand, Indonesia, Sri Lanka, Vietnam, Madagascar and Mali. The presenting symptoms and the clinical findings were typical of a malaria infection. The main problem in the future will be the increase in imported cases of malaria.
The haematological findings and case history of 3 patients with the association of acute myeloid leukemia and translocation involving the long arm of chromosome no. 11 are presented. The recipient chromosome for the translocated material from chromosome 11 differs in all the three cases being namely chromosomes 1, 10 and 17.
From 1981 to 1989, 12 patients of the University Hospital, Kuala Lumpur, were diagnosed to have Evans syndrome based on direct antiglobulin test (DAT) positive haemolytic anaemia and immune thrombocytopenia occurring either simultaneously (7 patients) or consecutively (5 patients). Their mean age at presentation was 24.8 years with a marked female preponderance. All 12 patients were given high dose steroid after diagnosis. Subsequently, other modalities including intravenous immunoglobulin (1 patient) and high dose methylprednisolone (1 patient) were given. Three patients died of intracranial haemorrhage during the first admission while 1 patient died of pulmonary embolism six months after diagnosis. Three patients had splenectomy because of thrombocytopenia. Six patients tested positive for antinuclear factor and antibodies to double stranded DNA and four of them died. Positive serology appeared to be associated with a poorer prognosis. Follow up observations indicate that patients who survive the acute attacks fare reasonably well.
Cerebral infarction in the young is likely to be non-atheromatous. While in previous studies no cause has been found in 40% to 50% of patients, an increasing role for haemorheological factors is becoming apparent. Among these, an association between antiphospholipid antibodies (aPLs) and ischaemic cerebrovascular disease is now well-recognised. This entity has not been previously reported in Malaysian patients. In a study of 80 patients with stroke below the age of 50 years who were seen at the University Hospital, Kuala Lumpur, between January 1982 and May 1992, 3 patients with ischaemic cerebral infarction were found to have aPLs. aPLs was detected using ELISA method for anticardiolipin antibodies (aCLs), and presence of lupus anticoagulant (LA) was established by kaolin clotting time, thromboplastin inhibition test and platelet neutralisation procedure. Only 1 patient had active systemic lupus erythematous. Cerebrovascular events were recurrent in one of the 2 non-lupus patients. aPL-related stroke should be considered in young patients who have cerebral ischaemia occurring without obvious cause. More cases are likely to emerge in Malaysia with active screening.
The management of haemorrhagic episodes in patients with factor VIII inhibitor is difficult and the outcome rather unpredictable. The use of an investigational drug, that is, activated recombinant factor VII (rFVIIa) in a young non-haemophiliac patient with spontaneous occurrence of factor VIII inhibitor who presented with life-threatening retroperitoneal haemorrhage is reported. There was prompt achievement of haemostasis with rFVIIa after the patient had failed conventional therapy with factor IX and Autoplex. Two further episodes of retroperitoneal bleeding again responded promptly to rFVIIa therapy.
Drug-induced agranulocytosis is a potentially fatal complication despite advances in supportive care. A patient with carbimazole-induced agranulocytosis associated with marked depletion of granulocytic precursors in the marrow was treated with granulocyte-macrophage colony stimulating factor (GM-CSF) at a dose of 5 micrograms/kg subcutaneously daily for one week. The absolute neutrophil count rose above 1 x 10(9)/L after one week of GM-CSF therapy. The GM-CSF probably expedited the recovery of the neutrophil count. Further studies are warranted to delineate the role of GM-CSF in the treatment of drug-induced agranulocytosis.