Archna Sharma

Unique Red Cell Abnormality in a Case of Fatal Methylene Blue Induced Haemolysis

Abstract The appearance of a single blob of denatured haemoglobin in red cells staining prominently with supravital dyes is described in a case of fatal haemolysis following infusions of methylene blue (MB) for the treatment of toxic methaemoglobinaemia. This unique inclusion hitherto unreported may be used to predict impending haemolysis.

Hematology morphology forum. Paroxysmal cold haemoglobinuria.

A 24 year old male presented with 3 days fever, chills, jaundice and weakness. The patient was conscious, with stable vital signs. He had moderate pallor, icterus, no lymphadenopathy or organomegaly. Other systemic review was normal. Investigations showed Hb 3.6 gm/dl, PCV 0.95 l/l, MCV 82 fl, MCH 33.2 pg, RDW 14.4%, WBC 7.8 £ 10 /l, lymphocytes 1.1 £ 10 /l, monocytes 0.27 £ 10 /l, neutrophils 5.3 £ 10 /l, platelet count 112 £ 10 /l, reticulocyte count 3.5%. The peripheral blood film showed anaemia and autoagglutination of red cells. There was erythrophagocytosis by the neutrophils as illustrated in Fig. 1 and significant red cell rosetting around neutrophils as seen in Fig. 2. Urine examination was positive for haemoglobin. Liver function test were normal except for a total bilirubin 9.8 mg/dl, direct bilirubin 0.4 mg/dl. Blood bank serology showed haemolysis in the serum, positive autocontrol, negative Direct Coomb’s test and Indirect Coomb’s test. The Donath Landsteiner antibody was demonstrable in patient’s serum. Bone marrow was not diagnostic. Tests for syphilis, retroviral studies, Hepatitis B surface antigen, HCV, Dengue, mycoplasma and infectious mononucleosis were negative.

Early markers of occult megaloblastosis for low-cost detection of hyperhomocysteinemia in patients with ischaemic stroke: preventive approach for primary health care

Recent studies have focussed on the association between elevated homocysteine levels with megaloblastic changes and thromboembolic events, but the relationship between occult megaloblastosis (with normal haemoglobin levels) and ischaemic stroke has not been widely explored. The objective of this study is to establish a simple and economical marker for the detection of occult megaloblastosis at the community health care level in developing countries. A hundred patients who met the inclusion criteria were studied. At the 5% level of significance, the levels of cobalamin and folate were significantly lower, while the number of hypersegmented neutrophils on the peripheral smear was higher in patients from Group A (70 patients with high homocysteine) compared with the patients in Group B (30 patients with normal homocysteine). Forty-five (64.2%) of the 70 patients in Group A showed hypersegmentation of neutrophils in the peripheral smear. The high cost and difficulty in performing the vitamin assays limit their use as early markers of megaloblastosis. Hence, we conclude that in developing countries, the detection of hypersegmented neutrophils can be used at the primary healthcare level for early diagnosis of occult megaloblastosis, so that early therapeutic interventions with vitamins can prevent attacks of hyperhomocysteinemia-induced ischaemic stroke.

Reversible pancytopenia caused by severe copper deficiency in a patient with Wilson disease

1 Bone marrow aspirate showing moderate dyserythropoiesis and cytoplasmic vacoulation in erythroid (red arrows) and myeloid precursors (blue arrows) A 26-year-old woman was referred to the haematologist for evaluation of new onset pancytopenia. She was diagnosed with Wilson disease at 13 years of age, when she developed extrapyramidal neurological manifestations, mainly dystonia, ataxia and tremors. Despite copper chelation therapy with zinc sulphate, she had disabilities due to dystonia and became wheelchair bound, requiring high level nursing care. She underwent clinical monitoring with yearly blood tests and her previous full blood counts were consistently normal; however, serum copper values were mildly reduced, consistent with those seen in Wilson disease. Her regular medications were zinc sulphate (200 mg thrice daily), benzhexol, baclofen, diazepam and lactulose. Her nutritional intake was good. Clinical examination revealed fixed posture deformities of upper and lower limbs, bilateral partial KaysereFleischer rings on ocular slitlamp examination, and there were no stigmata of liver disease.

DSP30 and interleukin-2 as a mitotic stimulant in B-cell disorders including those with a low disease burden

Chromosome abnormalities detected during cytogenetic investigations for B‐cell malignancy offer prognostic information that can have wide ranging clinical impacts on patients. These impacts may include monitoring frequency, treatment type, and disease staging level. The use of the synthetic oligonucleotide DSP30 combined with interleukin 2 (IL2) has been described as an effective mitotic stimulant in B‐cell disorders, not only in chronic lymphocytic leukemia (CLL) but also in a range of other B‐cell malignancies. Here, we describe the comparison of two B‐cell mitogens, lipopolysaccharide (LPS), and DSP30 combined with IL2 as mitogens in a range of common B‐cell disorders excluding CLL. The results showed that DSP30/IL2 was an effective mitogen in mature B‐cell disorders, revealing abnormal cytogenetic results in a range of B‐cell malignancies. The abnormality rate increased when compared to the use of LPS to 64% (DSP30/IL2) from 14% (LPS). In a number of cases the disease burden was proportionally very low, less than 10% of white cells. In 37% of these cases, the DSP30 culture revealed abnormal results. Importantly, we also obtained abnormal conventional cytogenetics results in 3 bone marrow cases in which immunophenotyping showed an absence of an abnormal B‐cell clone. In these cases, the cytogenetics results correlated with the provisional diagnosis and altered their staging level. The use of DSP30 and IL2 is recommended for use in many B‐cell malignancies as an effective mitogen and their use has been shown to enable successful culture of the malignant clone, even at very low levels of disease.