Try it
Pathology · Haematology: aplastic anaemia
Sample itemA 22-year-old man has fatigue, bruising and infections. Counts show pancytopenia with low reticulocytes, and there is no splenomegaly or lymphadenopathy. Marrow biopsy shows a hypocellular marrow with fat replacement and no abnormal cells. What is the most likely mechanism in most acquired cases?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. T-cell mediated autoimmune destruction of haematopoietic stem cells
Most acquired aplastic anaemia is idiopathic and is mediated by activated cytotoxic T cells that release interferon-gamma and TNF, causing destruction of stem cells. Treatment is stem cell transplantation for young patients with a matched donor, or immunosuppression with antithymocyte globulin and cyclosporine. Telomere defects and drugs such as chloramphenicol are other causes.
Option by option
A. Clonal ineffective haematopoiesis with a hypercellular marrow
Ineffective haematopoiesis occurs in myelodysplastic syndromes, where the marrow is typically hypercellular.
B. Peripheral destruction of cells by an enlarged spleen
Hypersplenism needs an enlarged spleen, and the marrow would be cellular.
C. T-cell mediated autoimmune destruction of haematopoietic stem cells (correct)
Correct. Autoimmune T-cell attack on progenitors leaves an empty fatty marrow.
D. Replacement of marrow by fibrous tissue
Marrow fibrosis occurs in myelofibrosis, with teardrop cells and a dry tap, and usually splenomegaly.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
More Pathology questions
- HaematopathologyBinucleate 'owl-eye' cells that stain for CD15 and CD30 are characteristic of which cond…
- NeuropathologyEosinophilic intracytoplasmic inclusions in the pigmented neurons of the substantia nigr…
- HaemodynamicsWhich of the following is NOT a component of Virchow's triad for thrombosis?
- Granulomatous diseaseNon-caseating granulomas with Schaumann bodies and asteroid bodies are typical of: