Lymphadenopathy: Plasmacytoma, MGUS and Lymphoplasmacytic Lymphoma – White Blood Cell Pathology

by Carlo Raj, MD

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    00:01 Our topic here is plasmacytoma.

    00:04 Plasmacytoma could be solitary, a lesion, consisting of once again monoclonal neoplasm to the plasma cell.

    00:11 So we have a monoclonal gammopathy.

    00:14 With solitary, one lesion would be perhaps in the bone marrow, intramedullary.

    00:22 Or if it’s extramedullary, the number one location for extramedullary type of plasmacytoma, solitary type, would be the upper respiratory tract.

    00:34 Solitary lesion type of plasmacytoma, bone marrow, extramedullary, upper respiratory tract.

    00:41 And you have bony plasmacytomas, usually eventually present into or lead into multiple myeloma 10-20 years down the road, okay? So you have your solitary lesion or solitary plasmacytoma which could be intramedullary or could be extramedullary.

    00:59 And then the bony plasmacytomas may then go on to multiple myeloma decades down the road.

    01:07 Extraosseous plasmacytomas rarely progress and can often be surgically cured.

    01:13 Okay, make sure that you know the different patterns of plasmacytomas as once again you have a monoclonal gammopathy from the plasma cell but it could be solitary.

    01:24 It could be within intramedullary, extramedullary.

    01:26 It could be bony or could be extraosseous, which could be surgically cured.

    01:34 Our topic here is MGUS, which stands for monoclonal gammopathy of undetermined significance.

    01:40 So yet, we have another monoclonal gammopathy.

    01:43 You have an M protein.

    01:44 Oh, boy.

    01:45 So this would rather seem like it would be multiple myeloma.

    01:50 You actually find IgG or IgA.

    01:52 But guess what? Luckily with MGUS, there are no other bony infiltrative type of symptoms in your patient.

    02:00 Okay, so there is no bone pain.

    02:02 And as far as signs, no pathologic fractures.

    02:06 There is no hypercalcemia, no hypocalciuria, so on and so forth.

    02:10 Found in 1% of people greater than 50.

    02:12 Incidence would increase with age.

    02:15 And only a small percentage may then go on to multiple myeloma.

    02:20 So MGUS, quite common, it is actually the most common monoclonal gammopathy.

    02:25 You might find an M protein and might find a gamma spike with IgG or IgA.

    02:30 But you will not find punched out lesions.

    02:32 That to you, ladies and gentlemen, should be MGUS, monoclonal gammopathy of undetermined significance.

    02:42 Here we have lymphoplasmacytic lymphoma, a plasma cell neoplasm.

    02:48 I asked you earlier to, when you go through this, compare and contrast lymphoplasmacytic lymphoma with multiple myeloma.

    02:56 Let’s do this now.

    02:57 Multiple myeloma is a plasma cell issue obviously, but the problem arises from the bone marrow, right? There is every possibility with multiple myeloma which arises from the bone marrow to enter the lymph node and look like a lymphoma.

    03:11 Don’t forget that ever.

    03:13 So just because you have a disease or a cancer that begins in the bone marrow, it doesn’t mean it would present as a lymphoma.

    03:20 It very well could.

    03:22 Here, however, with Waldenstrom macroglobulinemia, it actually begins in the lymph node.

    03:28 So it’s a lymphoplasmacytic lymphoma.

    03:32 What is a plasma cell primitively or what cell differentiated into a plasma cell? A B-cell.

    03:40 So if you’re thinking about a lymph node in which you have a cancer that is developing into a plasma cell, then you have to be referring to the B cell.

    03:48 Welcome to Waldenstrom.

    03:50 So Waldenstrom consists of neoplastic B cells.

    03:52 Where are you? In your lymph node.

    03:55 Not necessarily plasma cells, that secrete monoclonal IgM.

    04:00 Interesting, isn’t it? What’s the immunoglobulin that you’re producing here? IgM, Waldenstrom.

    04:07 IgM.

    04:10 I asked you earlier when we began to section of plasma cell dyscrasia about the size of your IgM.

    04:17 It’s the biggest immunoglobulin.

    04:19 It is a pentamer.

    04:20 And imagine your patient secreting abundant IgM into circulation or gets into circulation, all this IgM which is now aggregating.

    04:33 This is not good.

    04:34 It behaves like a thrombus.

    04:37 In other words, you’ve heard of hyperviscosity syndrome.

    04:40 Hyperviscosity syndrome, mind you, could also be found in multiple myeloma, but predominantly found with Waldenstrom.

    04:50 Kind of like when we did our DKA, diabetic ketoacidosis, predominantly found in type 1 as being uncontrolled but could also be found in type 2.

    05:00 Hyperviscosity syndrome predominantly found in Waldenstrom, could also be found in multiple myeloma.

    05:05 Keep that in mind.

    05:07 Especially because of IgM, the pentamer.

    05:10 Next, so if there is hyperviscosity, how is the patient presenting? Think of it as being thrombus formation everywhere.

    05:16 There will be visual disturbance.

    05:18 There will be neurologic issue, maybe stroke like symptoms, or there is going to be bleeding.

    05:23 Because of hyperviscosity, there is every possibility that your patient may then become thrombocytopenic because of thrombus formation.

    05:30 Keep that in mind.

    05:33 Now, with Waldenstrom macroglobulinemia, would you have such major bone lytic lesions? Not so much.

    05:41 Okay? Not so much.

    05:44 Patients also present with hepatomegaly, lymphadenopathy and anemia.

    05:50 The disorder is progressive and incurable to this point and unfortunately, we have another disease, a cancer here which is a lymphoma type.

    05:59 That once again has very, very poor survival.

    06:03 Now at this juncture, I kindly ask you to compare and contrast Waldenstrom with your multiple myeloma.

    06:11 You do that first as far as your priorities.

    06:14 Once you have understood and define differences between those two, then you take a look at monoclonal gammopathy of underdetermined significance and do not forget about plasmacytomas.

    06:25 The solitary type and what it means to be within the medulla and extramedulla.

    06:29 You go in that order and keep your thoughts nice and organized with plasma cell neoplasm.

    06:34 There is no way that the boards will trick you.

    06:37 If you just look at everything at once and start memorizing, things become a little bit more difficult, okay? A picture here is showing you Rouleaux formation.

    06:49 I want to tell you that this is a nonspecific finding.

    06:52 You’re not going to find this only with multiple myeloma.

    06:55 You’re not going to find this only with Waldenstrom.

    06:57 You could find it with a monoclonal gammopathy.

    07:00 So what’s happening? It’s physics, okay? So I’m not going to go into great detail.

    07:05 You’re not going to be asked about a question -- about the pathogenesis of Rouleaux formation, my goodness But be able to identify it and know where in terms of family of diagnosis you are.

    07:18 You see Rouleaux formation.

    07:21 In terms of family, you should be thinking about plasma cell dyscrasia.

    07:25 That’s it.

    07:26 Now, it’s because of the charges, just to make sure we’re complete.

    07:30 Because remember, Waldenstrom or multiple myeloma, you’re producing IgG.

    07:34 IgG is being produced by which plasma cell dyscrasia? Good, multiple myeloma.

    07:40 IgM, Waldenstrom.

    07:43 Point is, lots of immunoglobulin, you mess up the charges and the charges here are then going to attract RBCs, literally stacking up on top of each other like poker chips.

    07:55 Poker chips.

    07:56 Hence, it's called Rouleaux formation.

    About the Lecture

    The lecture Lymphadenopathy: Plasmacytoma, MGUS and Lymphoplasmacytic Lymphoma – White Blood Cell Pathology by Carlo Raj, MD is from the course Lymphadenopathy – White Blood Cell Pathology (WBC).

    Included Quiz Questions

    1. Upper respiratory tract
    2. Lower respiratory tract
    3. Ribs
    4. Bone marrow
    5. Thoracic and lumbar vertebrae
    1. Monoclonal gammopathy of undetermined significance
    2. Extramedullary plasmacytoma
    3. Lymphoma
    4. Multiple myeloma
    5. Solitary plasmacytoma of bone
    1. Punched out lesions
    2. Gamma spike with IgG
    3. M protein
    4. Gamma spike with IgA
    5. Monoclonal paraprotein band
    1. Waldenstrom macroglobulinemia
    2. Hodgkin's lymphoma
    3. Solitary plasmacytomas
    4. Non-Hodgkin lymphoma
    5. Multiple myeloma

    Author of lecture Lymphadenopathy: Plasmacytoma, MGUS and Lymphoplasmacytic Lymphoma – White Blood Cell Pathology

     Carlo Raj, MD

    Carlo Raj, MD

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