Intravenous immunoglobulin (IVIg) is a common therapy for patients with immune thrombocytopenia (ITP). The initial response rate for IVIg is 80%[1][1] and is typically rapid, with some patients responding in 24 hours, although usually in 2–4 days.[2][2] When IVIg is used alone, the response is

Primary polycythaemia, also known as polycythaemia vera (PV), is a myeloproliferative neoplasm (MPN) which is associated with arterial and venous thrombosis and which can contribute to significant morbidity and mortality if untreated. Arterial thrombosis accounts for a large proportion of PV-related thrombotic events which may manifest as stroke and myocardial infarction. There is an abundance of literature documenting thrombosis arising in the cerebral vasculature secondary to PV. However, vertebral artery thrombosis associated with PV has not been previously described. We present a case of vertebral artery thrombosis as the presenting manifestation of PV. This case demonstrates the importance of recognising MPNs as a cause of an unusual presentation of thrombosis.

The case of a patient with the aplastic variant of hairy cell leukaemia, successfully treated with the drug Deoxycoformycin(Pentostatin), is presented. It is very important to be aware of this rare variant of a rare disease so that the right treatment can be offered.

Hairy cell leukaemia (HCL) is a rare lymphoproliferative disorder associated with pancytopenia, splenomegaly and the presence of typical hairy B lymphocytes in the bone marrow and/or peripheral blood. The most significant complication relates to opportunistic infections that arise as a consequence of neutropenia and monocytopenia. HCL is occasionally associated with systemic autoimmune disorders including polyarteritis nodosa and rheumatoid disease. Secondary autoimmune haemolytic anaemia (AIHA) appears to be rare. We report on two cases of HCL complicated by fatal cold anti‐i AIHA. Fulminant haemolysis causing death is rare in cold AIHA and only a few individual cases have been reported, none having anti‐i specificity.

M Elia's commentary1Elia M Oral or parenteral therapy for vitamin B12 deficiency.Lancet. 1998; 352: 1721-1722Summary Full Text Full Text PDF PubMed Scopus (98) Google Scholar reviews the use of parenteral and oral vitamin B12 preparations; but does not discuss the rare but difficult management of hypersensitivity to these preparations. We report the case of a 73-year-old woman who presented to our department in 1992 with a macrocytic anaemia and a reduced serum concentration of vitamin B12 of 109 ng/mL (normal range 170-740 ng/mL). Other haematinic concentrations were normal and she was positive for antigastric parietal cell antibodies, but negative for antigastric intrinsic factor antibodies. Treatment with intramuscular hydroxocobalamin 1000 μg was started; 14 h after the injection, she became flushed and developed facial swelling. Subsequent treatment with one 50 μg tablet of cyanocobalamin was followed by general malaise, facial flushing and swelling, vomiting, and hypertension, within 2-3 h and at 6 h she had developed an extensive, weeping dermatitic eruption affecting her face. She was treated with intravenous chlorpheniramine and intravenous and topical corticosteroids. Skin patch testing confirmed sensitivity to common allergens but not to colbalt. Bone marrow and cytogenetic studies done after treatment with vitamin B12 excluded myelodysplastic syndrome. The initial treatment with intramuscular and oral vitamin B12 was sufficient to maintain normal serum concentration until early 1995, when it fell to 139 ng/L. She was subsequently managed with intermittent blood transfusion that was also temporarily successful in restoring her serum vitamin B12 to within normal range and correcting the macrocytic anaemia to varying degrees. However, by early 1998, this approach had become ineffective and her serum vitamin B12 had fallen to 100 ng/L. In view of anaemia and potential neurological difficulties, a decision was made to treat her with intramuscular hydroxocobalamin with corticosteroid and antihistamine cover. On the intensive-care unit, she received a test dose of 100 μg after premedication with intravenous hydrocortisone 200 mg, chlorpheniramine 10 mg and ranitidine 150 mg. The absence of a reaction made possible the administration of a full therapeutic dose of intramuscular hydroxocobalamin 1000 μg. Subsequently, the serum vitamin B12 increased to within the normal range and the macrocytic anaemia has resolved. Hypersensitivity reactions to vitamin B12 preparations2Denis R Amin S Cummins D Sensitivity reaction to parenteral vitamin B12: recurrence of symptoms after Marmite ingestion.Clin Lab Haemat. 1996; 18: 129-131Crossref PubMed Scopus (3) Google Scholar are rarely documented and range from urticarial and dermatitic rashes to circulatory collapse and death. A sensitivity to parenteral and oral preparation has been previously reported, and there is also a report of a sensitised patient reacting to Marmite, which is fortified with cyanocobalamin. Our patient is the first report of successful prevention of vitamin B12 hypersensitivity. We suggest that such patients might be managed similarly with premedication with hydrocortisone and antihistamine and close overnight inpatient observation.

Five out of nine adults (55%) with lymphoblastic disease developed severe avascular necrosis of bone (AVN) when treated with a Berlin-Frankfurt-Munster (BFM) ALL protocol similar to the current joint MRC-ECOG ALL trial (UKALL XII). The principal purpose of these intensified regimens is to improve long-term disease-free survival without necessarily increasing toxicity and secondary morbidity. The presentation of all five was non-specific bone pain occurring after the re-intensification block of chemotherapy containing high doses of dexamethasone. Three types of diagnostic imaging were performed and magnetic resonance imaging (MRI) proved superior in demonstrating AVN and showed it at an earlier stage than plain radiographs or isotopic scans. We believe that the dose of corticosteroids was the major factor in the development of AVN. The five men in our series all remain in first remission with a median disease-free survival of 3.5 years (range 2-8 years) but with varying degrees of disability due to AVN. Clinicians involved in UKALL XII and similar trials should be aware of this debilitating and potentially crippling complication when using high-dose steroid-containing regimens, perform MRI scan early and modify treatment if necessary.

A patient with ANLL FAB subtype M1 was found to possess a t(16;21)(p11;q22) and trisomy 10. The 16;21 translocation has been reported in 12 other cases of ANLL, of various subtypes, and its relationship to the disease profile is discussed.

Two classical autoimmune polyendocrine deficiency syndromes with heritable tendencies are described, Type 1 diabetes mellitus being associated with the Type 2 polyendocrine deficiency syndrome (Schmidt's syndrome). A man with Type 1 diabetes mellitus is described who developed an unusual combination of five autoimmune conditions (myasthenia gravis, Addisonian pernicious anaemia, adrenalitis and thyroiditis) which did not fit into the Type 1 or Type 2 classical polyendocrine deficiency syndromes. This suggests that the autoantibody, biochemical and haematological screening of affected individuals and their relatives should be extended to anticipate a wider range of potential autoimmune conditions.