Plasmablastic Lymphoma and Multicentric Castlemans Disease

Clinical context

37-year-old man who is HIV positive

At a glance

StainMGG

About Plasmablastic Lymphoma and Multicentric Castlemans Disease

This is a great blood film - assuming it's not yours, that is. This patient is HIV positive with a history of an immune dysregulation syndrome called Castlemans disease. What they actually have is a combination of plasmablastic lymphoma, and Castlemans disease. This patient had multicentric Castlemans with a massively elevated IL-8. He was actually KSHV negative (KSHV/HHV8 is associated with MCD). You should be able to get plasmablastic lymphoma by morphology but the Castlemans is a bit niche. 

The blood film is full of abnormal cells. There are immature lymphoid cells - morphologically they are blasts but the flow suggested they were lymphomatous, so expressing some markers of maturation. There are abnormal monocytes, which are extremely reactive and could be consistent with either diagnosis. There are immature plasma cells - plasmablasts - with deep dark cytoplasm laden with abnormal Immunoglobulins and which again feed the immune dysregulation. As part of the bone marrow infiltration, there are nucleated red blood cells. Surprisingly, the myeloid series is relatively normal. There are neutrophils, which are reactive. There is a significant thrombocytopenia with platelet anisocytosis. Whether this is bone marrow failure or an associated immune ITP-like destruction we never found out. The red cell morphology is surprisingly pretty normal, apart from an anaemia. There is some mild RCAP, and a few acanthocytes and reticulocytes but this does not look at all like a primary bone marrow / red cell disorder.

Overall, the extreme reactive change and plasmablasts should be making you think plasmablastic lymphoma. It couldn't really be plasma cell leukaemia - plasma cells are by definition mature cells, and these by morphology and flow are highly immature. It could be another form of unusual leukaemia - but if you've covered LPD and described plasma-cell type features, then you've got off to a great start.

Morphological features

Plasmablasts

Plasmablasts - Plasmablastic Lymphoma and Multicentric Castlemans Disease

This malignancy is characterised by a mix of highly reactive cells, and highly malignant ones such as these plasmablasts. These are immature plasma cells.

Nucleated RBC

Nucleated RBC - Plasmablastic Lymphoma and Multicentric Castlemans Disease

This is an nRBC - and is in keeping with the bone marrow infiltration causing a leucoerythroblastic picture.

Reactive Mooncyte

Reactive Mooncyte - Plasmablastic Lymphoma and Multicentric Castlemans Disease

This is a highly reactive monocyte which has likely phagocytosed red cells coated in antibody - the large white vacuoles. This is called emperipolesis, and while this is prominent in PCH it can be seen in some viral infections and extreme reactive conditions

Lymphoid Blast

Lymphoid Blast - Plasmablastic Lymphoma and Multicentric Castlemans Disease

This cell is a malignant cell - but the fine vacuolation makes it more likely to be a lymphoid blast over and above a plasmablast.

Plasmablasts

Plasmablasts - Plasmablastic Lymphoma and Multicentric Castlemans Disease

These cells with high NC ratio, extremely blue cytoplasm and involuted nuclei are plasmablasts.

Reactive Monocytes

Reactive Monocytes - Plasmablastic Lymphoma and Multicentric Castlemans Disease

These monocytes are all highly reactive - the extreme vacuolation tells the story that the patient is not at all well

Reactive Neutrophils

Reactive Neutrophils - Plasmablastic Lymphoma and Multicentric Castlemans Disease

The reactive nature of the white cells extends into the myeloid series. These neutrophils show toxic granulation.

Platelet Anisocytosis and Clump

Platelet Anisocytosis and Clump - Plasmablastic Lymphoma and Multicentric Castlemans Disease

There is a thrombocytopenia, with platelet anisocytosis and small platelet clumps.

Mild Red Cell Anisopoikilocytosis

Mild Red Cell Anisopoikilocytosis - Plasmablastic Lymphoma and Multicentric Castlemans Disease

Surprisingly, there isn't much to say about the red cells. There is some mild RCAP, and a few acanthocytes, but not much else.

Acanthocyte

Acanthocyte - Plasmablastic Lymphoma and Multicentric Castlemans Disease

There are a few of these cells - with spiky projections. There aren't enough to call them EDTA change. It's probably renal failure associated.

Reticulocyte with Pappenheimer bodies

Reticulocyte with Pappenheimer bodies - Plasmablastic Lymphoma and Multicentric Castlemans Disease

This reticulocyte has a few faint pappenheimer bodies. This all fits with a very unhappy bone marrow and a significant anaemia.

Plasmablast with Prominent Golgi zone

Plasmablast with Prominent Golgi zone - Plasmablastic Lymphoma and Multicentric Castlemans Disease

Answers on a postcard for what this cell is. It's a plasmablast, but it has a golgi zone. Plasma cells don't get Golgi's until they are mature. Promyelocyte version of a plasmablast? Probably bad news, whichever way.

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