6040BMS Coursework – Presentation Cases
Case 1
A 61-year-old woman with Rai Stage-III CLL was initially treated with four monthly cycles of
fludarabine. Because of an unsatisfactory response, she then received three cycles of standard
chemotherapy followed by radio-immunotherapy (RIT) supported with autologous Peripheral
Blood Progenitor Cell (PBPC) rescue.
Two days after her PBPC infusion, the patient was admitted to a local hospital for culture-negative
neutropenic fever and treated with IV antibiotics and G-CSF. During this hospitalisation, nonirradiated blood components including 2 units of non-WBC-reduced RBCs and 3 units of WBCreduced single-donor apheresis platelets were transfused over two days. The patient was
discharged to home after 7 days in the hospital, afebrile and with a normal WBC count.
Ten days after transfusion of the PBPCs, the patient presented with a 2-day history of recurrent
fevers and a new, salmon-coloured, diffuse, maculo-papular rash. On admission, laboratory
studies revealed a WBC count of 6.1×109/L with an absolute neutrophil count of 5.4×109/L, an Hb
level of 80 g/L, and a platelet count of 16×109/L. Diarrhoea, liver function test elevations, and
pancytopenia developed over the next 4 days. Skin biopsy, performed the day of admission,
showed a predominantly mononuclear cell infiltration (see Figure) and a marrow biopsy revealed
marked hypocellularity.
Figure showing a marked mononuclear cell infiltrate on a skin biopsy.
Based on the above information, provide an initial diagnosis of this condition, further examinations
that would be required to confirm the diagnosis and a potential approach to therapy.
Case 2
Haemolytic Disease of the Foetus and Newborn (HDFN) is most commonly caused by IgG antiRhD antibodies generated in RhD negative mothers who have been immunised against RhD
antigen through previous pregnancies or blood transfusion.
Your Laboratory has been sent samples from a pregnant 28 year old woman requesting a blood
group and antibody screen after an ultrasound scan at 29 weeks of gestation suggested the
presence of severe foetal hydrops in the foetus, a clinical feature normally associated with HDFN.
The mother’s blood group is O RhD positive. She had given birth to a single child two years
earlier, who exhibited evidence of jaundice at birth. The newborn required a blood transfusion on
day 3 of life and subsequently died on day 7.
Describe in detail how you would investigate this case further and describe any therapeutic
measures the child may require.
Case 3
A 2-year-old boy with no known medical illness weighing 11.6 kg was admitted for multiple
bruises for a one-week duration due to thrombocytopenia. A diagnosis of immune
thrombocytopenic purpura was made.
He was started on intravenous immunoglobulin at 1 mg/kg dose and indicated for two units of
whole blood (WB) derived platelet transfusion in view of bleeding manifestation and very severe
thrombocytopenia with platelet level of 3 × 109/L. The patient is B Rhesus D (RhD) positive and
received O RhD positive WB derived platelets due to the unavailability of blood group B platelets.
He developed chills, rigours and shortness of breath during the second unit of platelet transfusion.
He deteriorated with oxygen saturation of 80% under room air, hypotensive (94/56 mmHg),
tachycardia (156 beats/minute), and tachypnea (26 breath/minute) with a temperature of 38 ◦C.
His haemoglobin (Hb) dropped to 8.2 g/dl from 10.2 g/dl supported with blood film evidence of
spherocytosis and small agglutinations . He had slight jaundice and elevated lactate
dehydrogenase (LDH) levels of 1209 U/L. His erythrocytes demonstrated a positive Direct
Antiglobulin Test (DAT).
Suggest a potential diagnosis of the condition manifested, how you could confirm this diagnosis
and indicate a possible cause for these symptoms.
Case 4
A 34 year old woman was admitted to hospital for a planned caesarian section to deliver her child
at 41 weeks of gestation. A healthy baby was born but 8 hours postpartum, she complained of
severe abdominal pain and she showed a fall in haemoglobin level (129 to 74 g/L) and she was
rushed to surgery to repair an intrauterine bleed.
One hour before surgery, the patient received a transfusion of one unit of leukocyte-reduced red
blood cell concentrate (RBC), and intra-operatively she received three units of RBCs and four
fresh frozen plasma (FFP) units. Near surgery completion, during transfusion of the fourth FFP
unit, the patient became hypoxic. Arterial oxygen tension dropped from 100% to 82%, clear
frothy sputum emerged from the endotracheal tube, and systemic blood pressure decreased
from 140/100 to 90/40 mmHg. Echocardiography within one hour after onset of symptoms
revealed normal left ventricular function. A chest radiograph showed extensive bilateral lung
infiltrates (Figure 1), suggesting acute respiratory distress syndrome (ARDS).
Two hours before the first transfusion, the neutrophil count was 12.0 x 109/L (normal range 1.5-7.5
x 109/L), and it dropped to 0.38 x 109/L at 4 h after onset of symptoms. Simultaneously, the
platelet count dropped from 164 x 109/L to 59 x 109/L, and the monocyte count dropped
from 0.84 x 109/L to 0.00 x 109/L.
She had normal body temperature (35.6-36.9 0C) and infection was ruled out, with
negative cultures of blood, urine, and endotracheal secretion. The patient’s condition improved
gradually; after 41 h, she was extubated, but required oxygen support for an additional
60 h. Five weeks later, a chest radiograph showed complete regression of infiltrates and no signs
of sustained lung injury.
Suggest a potential diagnosis of the condition affecting the patient, how you could confirm this
diagnosis and indicate a possible means to minimise the risk of this condition arising.