Tag Archives: Case Reports

Castleman’s Disease

  • General Information

    • Lymphoproliferative disorder which is histologically characterized by angiofollicular lymph-node hypertrophy.
    • In the differential diagnosis for localized/diffuse lymphadenopathy with or without systemic manifestations.
    • Rare and relatively benign disorder which can mimick lymphoma clinically, but varies from the latter histologically, prognostically and in its treatment options.
  • Types

    • Localized CD:
      • By definition, localized to one site.
      • Features lymphoid hyperplasia associated with excessive angiogenesis.
      • Asymptomatic in over 50% of patients and is often discovered incidentally.
      • Histological diagnosis requires lymph-node biopsy.
    • Multicentric CD:
      • Characterized by a predominantly lymphadenopathic presentation consistently involving peripheral lymph-nodes and manifestations of multisystem involvement.
      • Considered as a systemic B cell lymphoproliferation, probably arising in immunoregulatory deficit, and resulting in the outgrowth of clonal B-cell populations.
      • It is always symptomatic.
      • Symptoms, primarily a consequence of elevated Interleukin-6 (IL-6) production, are:
        • Asthenia(65%)
        • Weight loss(67%)
        • Fever(69%)
        • Polyadenopathy is common with a mean of four sites involved and is often associated with hepatosplenomegaly.
        • Histological diagnosis is made upon biopsy of an excised peripheral lymph-node.
    • Other forms/associations:
      • A POEMS (Peripheral polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy(M-Protein) and Skin signs) syndrome is observed in 24% of patients.
      • Some MCD forms are associated with Kaposi’s sarcoma displaying prominent vascular proliferation and characteristic lesions.
      • MCD associated with human immunodeficiency virus(HIV) infection is very similar to MCD observed in non-HIV-infected patients, except for the high prevalence of pulmonary symptoms and for the stronger association with Kaposi’s sarcoma.
        • Progression to malignant lymphoma in MCD associated with HIV is frequent.
        • Within a prospective cohort study of HIV-infected patients with MCD, 23% developed HHV 8-associated non-Hodgkin lymphoma.
  • Etiology

    • Poorly understood and no genetic or toxic factor has so far been identified.
    • The hypothesis of a viral infection has been raised and several studies have suggested the role of human herpesvirus 8 (HHV-8), already implicated in Kaposi’s sarcoma. There are a couple of theories:
      • (i) the opportunistic presence of HHV-8, favoured by immune pertubations.
      • (ii) the direct pathogenic role of HHV-8, in association with dysregulation of cytokines.
      • Recent studies support the latter hypothesis by demonstrating that HHV-8 is able to produce an IL-6 homologue, the interleukin reponsible for the plasmacytosis and hypergammaglobulinaemia seen in MCD.
  • Diagnosis

    • Tissue is the issue!
    • Histopathology is characterized by distinctive follicles with expanded mantle zones of small lymphocytes forming concentric rings surrounding 1 or more atretic germinal centers.
    • There is prominent vascularity of the germinal centers, often with a single prominent penetrating vessel. Another important feature is vascular proliferation between the follicles, often with perivascular hyalinization.
    • The “onion-skinning” of mantle zone lymphocytes, together with the prominent central vessel, has been likened to the appearance of a lollipop
  • Management

    • Localized CD is treated by surgical excision which allows full recovery without relapse in almost all cases.
    • Multicentric CD:
      • No therapeutic consensus exists for MCD and diverse treatments (surgery/corticotherapy/chemotherapy) are used, often in combination.
      • Anti-interleukin-6 antibody has also been successfully tried in the alleviation of systemic manifestations.
      • The five-year survival rate in MCD is 82% and this prognosis appears to be far better than that encountered with malignant lymphomas.

Journal of Medical Case Reports 2007, 1:78

Advances in Anatomic Pathology 2009, 16: 4

Detailed Guide: Castleman Disease. American Cancer Society. 2011;http://www.cancer.org/Cancer/CastlemanDisease/DetailedGuide/.

How to write a case report!

JJSC

By Jeremy Jones 

First, let me be honest, a case report, even in the most prestigious of journals is unlikely by itself to help you land your first-choice fellowship in the Ivy Leagues. With that being said, I have found that the process of writing a case report or abstract can act as a stepping stone to more substantial publications not to mention helping to bolster your CV.

  1. Find an interesting case.

In all honesty, if you work at a hospital for longer than a week interesting cases will find you, but the point is the same—keep your eyes open for rare diseases and when you find them jot down the pertinent patient information for safe keeping. As an intern, I actually made a special list in Epic called “Cool Cases.” Anytime I took care of a patient that had a particularly rare disease or more commonly a rare manifestation of disease I would add their MRN to my list. Depending on the rotation you will have a variable amount of free time to write so if you can’t muster the strength to write during your Parkland MICU month (this is a joke; don’t do this) at least you will have the patient’s information so that you can reference later when you start writing.

One thing that you will realize very early is that hospital patients are on the whole very sick. They have the most complicated diseases generally at end or near end stage at the time of presentation. While this is an unfortunate reality, it provides us the opportunity to observe first-hand the natural history of many diseases.

  1. Start writing.

I think one of the hardest parts of writing during residency is getting started. Once you have started, you realize that it is not actually that hard or time consuming. The best way to get through this writer’s block is to realize that writing a case report is really just like writing a detailed H&P. In that vein, try not to spend more than an hour or so writing the initial presentation and facts of the case.

  1. Research the topic. (This step is actually interchangeable with the previous step)

Do a PubMed search on whatever topic you are writing about and read a few articles about the disease you are writing about. A helpful tip here is to get endnote (free through the UTSW lib) and learn how to use it. There is nothing more annoying than finishing a paper and spending weeks trying to find and edit citations before you can submit. Overall don’t spend more than a day or so reading papers before you start writing.

  1. Pick a journal/conference to submit.

It is helpful to look through journals online submission guidelines prior to finishing up writing because most of them will have different formats for their specific journal. For example, some will want key teaching points prior to the case/discussion, some want a general discussion of the disease prior to the specific case presentation and nearly everyone has a different style of citation (this is where it EndNote comes in handy).

  1. Finish writing.

Now that you know what format you need to write and you have done some research, make a few paragraphs (in total you are shooting for 2 or 3 pages max) explaining why your case is unique, what it does to advance medical literature, how smart you are or anything that you want to add.

  1. Don’t be afraid to collaborate.

This is one of the most important and probably least used tips I can offer. Your case report will be no less impressive if you have 2 authors as opposed to 1. There have been a number of times where I have written a paper and either lost interest or run out time. If you stop at this point all of your hard work was for nothing. A better option is to talk to one of your colleges, tell them about the case and ask them if they would like to help you finish editing and submitting the paper. This is really a win-win situation for both parties and will result in far more publications for everyone.

  1. Submit and wait.

If you have made it this far the hard work is done. Congratulations. All you need to do now is upload the article into whatever journal you picked and they will walk you through the process from there. You will have to sign a form saying that you have no conflict of interests and that you were involved in the writing of the article, etc. Most journals will give you a decision within 4 weeks or so but other will be much sooner or much, much later (ie 8 wks+).

An Example: http://www.clinical-breast-cancer.com/article/S1526-8209(13)00243-7/abstract