Muhajir Mohamed

Massive intravascular haemolysis after high dose intravenous immunoglobulin therapy.

A 58-year-old Caucasian female was known to have had autoimmune thrombocytopenia purpura (ITP) since childhood and had undergone splenectomy when she was 16 years old. She presented with a platelet count of 15 x 109/l with bleeding manifestations and high dose intravenous immunoglobulin (IVIG) was therefore administered. The IVIG administered was Intragam-P (CSL Ltd., Parkville, Victoria, Australia). Her platelet count showed good improvement and had normalized by 48 h. However 48 h after a second dose of IVIG, the patient complained of reddish urine and her haemoglobin concentration (Hb) was found to have dropped from 122 g/l to 100 g/l, falling to 80 g/l after a further three days. Severe haemolysis was evidenced by polychromasia, spherocytes and nucleated red cells in her blood film (top). High lactate dehydrogenase (LDH), high bilirubin and low haptoglobin levels were also noted. A direct antiglobulin test (DAT) was strongly positive (4 + ), anti-IgG was strongly positive (4 + ) and anti-C3d was negative. Anti-A antibody was eluted from the red cells. The plasma was dark red in colour due to intravascular haemolysis (bottom left). Urine was also red in colour due to haemoglobinuria (bottom right), with no red cells on urine microscopy. There was no renal impairment. The patients blood group was A RhD-positive. Tests for antinuclear antibody (ANA), anti-double stranded DNA and anti-extractable nuclear antigen were negative.

Early Application of High Cut-Off Haemodialysis for de-Novo Myeloma Nephropathy is Associated with Long-Term Dialysis-Independency and Renal Recovery.

Background Multiple myeloma (MM) is a haematological malignancy associated with kidney injury resulting from cast nephropathy, which can be caused by monoclonal free light chains (FLC). It has been demonstrated that early reduction of FLC can lead to a higher proportion of patients recovering renal function with a better outcome, especially if high cut-off haemodialysis (HCO-HD) combined with chemotherapy is used. Patients and Methods In this study, four cases with MM nephropathy were treated with HCO-HD and chemotherapy at a single institution during the period from August 2009 to August 2011. All of the patients presented with acute renal failure and high serum FLC. All patients underwent a bone marrow biopsy to confirm the diagnosis of MM, according to the WHO criteria. Three patients had de novo MM and one patient had relapsed light chain myeloma disease. All patients underwent HCO-HD concomitantly with specific myeloma therapy once the diagnosis or relapse of MM was established. Results After a medial follow up of 26 months, (range, 13–36) our data showed that all patients had a significant decrease in serum FLC through HCO-HD, proving the effectiveness of HCO-HD in managing MM. De-novo MM patients restored their renal function and achieved low-level FLC early in the treatment and became dialysis-independent. One patient with relapsed myeloma remained dialysis-dependent. Conclusion In summary, our study suggests that in myeloma nephropathy associated with light-chain MM, HCO-HD should be initiated as early as possible. At the same time a specific MM treatment should be initiated to gain control of the disease and salvage the kidneys in order to achieve dialysis-independency. Further randomized trials to confirm our results are warranted.

Gelatinous transformation of bone marrow in a patient with severe anorexia nervosa

An 18-year-old Caucasian female was referred by her general practitioner for evaluation of chronic anaemia.

Quinine-induced severe thrombocytopenia: the importance of taking a detailed drug history.

Drugs can lead to severe life-threatening thrombocytopenia. The mechanisms of drug-induced thrombocytopenia are increased destruction by immune-mediated platelet destruction or decreased platelet production by bone marrow suppression. Quinine is a drug used for the treatment of malaria and nocturnal leg cramps and is also an important ingredient in some herbal preparations. Quinine can very rarely cause thrombocytopenia by immune-mediated platelet destruction. In a patient with thrombocytopenia, a detailed history of all the medications including over-the-counter medications and herbal preparations is very important.

Bleeding associated with acquired factor V inhibitor in a patient on warfarin treated successfully with prednisolone

An 85-year-old man on warfarin for atrial fibrillation presented with skin bleeding. International normalised ratio (INR) and activated partial thromboplastin time (APTT) were elevated and did not correct even after warfarin reversal with vitamin K, prothrombin complex concentrate (PCC) and fresh frozen plasma. Mixing coagulation studies with normal plasma suggested the presence of an inhibitor rather than the multiple coagulation factor deficiencies expected with warfarin. Assays of the common-pathway coagulation factors revealed factor V concentration <2% with inhibitor level elevated to 11 Bethesda units. The bleeding resolved following a course of corticosteroids. Coagulation studies and factor V level returned to normal along with resolution of the inhibitor. We report the case of the diagnostic dilemma posed and successful therapy implemented despite the limited evidence-based data being available for the treatment of this rare condition.

Diagnostic and Therapeutic Quandaries in a Patient with Primary Hepatic Lymphoma and Concurrent Hepatitis C Infection

Primary hepatic lymphoma (PHL) is a very rare sub-type of non-Hodgkin’s lymphoma and hepatitis C infection may be a contributory factor. The association of hepatitis C infection and PHL causes difficulties in management since safety of rituximab in such situations is unclear due to lack of evidence. The role of anti-viral therapy in combination with chemotherapy is also uncertain. We describe the diagnostic and therapeutic challenges posed by a patient who was diagnosed with PHL and concurrent hepatitis C infection.

Non-Hodgkin lymphoma manifesting as massive malignant chylothorax: successful management with chemotherapy and ambulatory drainages using indwelling pleural catheter.

Recurrent cancer‐related chylothorax is generally managed by talc pleurodesis or indwelling pleural catheter in the palliative care setting to relieve symptoms and improve quality of life. In chylothorax associated with curable/treatable malignancies like lymphoma, there are scarce data regarding the efficacy and safety of indwelling pleural catheters. We report a case of recurrent massive chylothorax associated with non‐Hodgkin lymphoma who demonstrated long‐term remission of lymphoma and complete regression of chylothorax after treatment with combination chemotherapy and ambulatory drainages using indwelling pleural catheter.

A man with acute venous thromboembolism and thrombocytopenia

A 64 year old man presented to the emergency department with shortness of breath and pleuritic chest pain of 12 hours’ duration. Eight days earlier he had undergone radical prostatectomy for early stage prostatic cancer. He had been given tramadol for analgesia and unfractionated heparin (5000 units subcutaneously) twice a day for four days for postoperative thromboprophylaxis. His full blood counts and biochemical parameters were within normal limits and he was discharged home on the fourth day after surgery with no complications. His medical history and family history were unremarkable. On examination his respiratory rate was 25 breaths/min, heart rate was 100 beats/min, blood pressure was 130/88 mm Hg, and oxygen saturation in room air was low (90%). Cardiac, respiratory, abdominal, and neurological examinations were normal. His left leg and thigh were swollen but non-tender. There were no bleeding manifestations or evidence of active infection at that time. Chest radiography and electrocardiography were unremarkable. However, axial computed tomography pulmonary angiography showed extensive thromboemboli in the right and left main pulmonary arteries extending more distally and associated with bilateral wedge shaped pulmonary infarcts (fig 1⇓). Doppler ultrasound showed thrombosis in the left popliteal vein extending up to the common femoral vein. Full blood counts showed low platelets (34×109/L), normal haemoglobin, and normal numbers of white cells. Blood film examination was unremarkable. His coagulation assays, renal parameters, and liver function tests were within normal limits. Fig 1 Axial computed tomography pulmonary angiogram showing extensive thromboemboli in the right and left main pulmonary arteries (yellow arrows) extending more distally and associated with bilateral wedge shaped pulmonary infarcts (blue arrowheads) ### 1 What is the most likely diagnosis in this patient? #### Short answer Heparin …

Multifocal primary bone lymphoma: durable complete remission after R-CHOP chemotherapy

Primary bone lymphoma (PBL) is a type of non-Hodgkins lymphoma predominantly affecting the skeletal system. PBL is an extremely rare cancer in adults affecting mainly the axial skeleton. The extent of bone involvement in these patients is variable. Most of the cases reported had single or a few skeletal lesions. We report a patient who had extensive multifocal lymphoma involving the axial skeleton and a very good and durable response to R-CHOP chemotherapy.

Ring chromosome with deletion 7q in acute myeloid leukaemia

Cytogenetic abnormalities can be detected in approximately 50–60% of newly diagnosed adult patients with acute myeloid leukaemia (AML). Monosomy of the chromosome 7 (−7) and deletion of the long arm of the chromosome 7 (7q–) are considered as high cytogenetic-risk AML with a poor prognosis. These abnormalities can occur, as a single chromosomal aberration, in approximately 8% of newly diagnosed AML. We report an elderly patient with AML who had deletion 7q (7q–) along with ring chromosome, which was demonstrated in conventional cytogenetics and fluoresecent in-situ hybridisation techniques.