Concluded Case

Rosai- Dorfman disease

18 yr old ,girl,college student,from neighbouring state,presented with painless swelling rt side of neck since 2 yrs Chief Complaints She developed neck swelling rt side 2 yrs ago Initially only single nodule ,slowly, the number of nodules increased.Denied having pain ,loss of weight or appetite / fever.The swelling is remaining static for the last 1 yr. Evaluated at her home town in different hospitals,biopsy done twice. After the biopsy she was informed that there is no further treatment available .She has no primary neurological problem.The reason to visit in the OPD is asking for a help to ref her to a proper doctor for further possible management at least for cosmetic reason. History No significant medical illness in the past.No family history of any type of malignancy .No history of TB in the family. Vitals Afebrile. BP110/ 60 mmhg.HR76/ mt. Physical Examination The only abnormality in physical exam is multiple mobile lymph nodes,varying in size,2 to 4 cm in the rt side of neck including submandibular lymph node ,some of them are matted together.No tenderness noted. No other lymphnode noted. No hepato-spleenomegaly noted. Investigations All blood work up including peripheral smear ,bone marrow,blood biochemistry,immunoelectrophoresis were normal.Ct chest and abdomen were normal. Cervical lymphnode biopsy done ,this is the3rd biopsy. Diagnosis 1st two biopsy report: Rosai Dorfman disease. Management Management waiting for the biopsy report. Question : What is the possible diagnosis ?


Concluded answer

Thanks Curofy and all doctors who answered the case. Clinical discussion Non- Hodgkin Lymphoma to exclude.Surgeon's opinion for Non Hodgkin Lymphoma.Hematologist and oncologist for a benign rare disorder. Asymptomatic ,remaining static for one year and no increase in size of the glands are the points for discusdion. Surgery done Submandibular and big cervical nodes were removed. Histopathology.Lymph node architecture is completely distorted Marked dialatation of lymphatic sinuses with numerous lymphocytes and histiocytes,plasma cells with fine vacules with vesicular nucleus and vkear cytoplasm.Phagocyted lymphocytes also noted - " emperipoleses". Immunohisyochemistry : Cells were + ve for protein S100,antitrypsine. CD 30 - ve CD 3 + ve in T cells. CD 20 + ve in B cells Final Diagnosis : Rosai- Dorfman disease.

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I guess lymphoma still needs a consideration here, what abt other LN areas or Cxr, HIV status for her

Apparently swellings are hard nodular and matted so primary suspicion is TBAdenitis Your workout proved it is a c/o ROSAI DORFMAN DISEASE So we will like to look deep insight in the disease kindly enlighten and guide the treatment Thanx for nice sharing

Thanx dr Dinesh Gupta

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Rule out Tb lymphadenitis as some lympnodes are matted . If Rossi foreman disease is the final diagnosis . It is accumulated histocytes in the lymphnodes .Cervical area is the common site . It can be autoimmune disorder associated with viral infection . Management parts includes chemotherapy . Drugs like methotrexate are given .Vinblastine or thalidomide can be alternative. Usually does not threaten the life.

Its an uncommon dease. Any pet scan done to rule out lymphoma madam

This is tubular lymphoma...need pulmonologist opinion

Congratulations! Your case has been selected as Case of the day and you have been awarded 5 points for sharing the case. Keep posting your interesting cases, Happy Curofying!

Rare disorder. Can you show the whole histopath report. Is this an excisional or core needle biopsy. As RDD is rare, I think the diagnosis should only be made by an experienced hematopathologist. Clinically + the path report you have put up fits with the diagnosis. RDD can co-exist with lymphomas, or one can arise later, but as this patient has relatively stable LN I think this is unlikely in her, Management depends on symptoms and patient preference. Usually we do not treat this is asymptomatic patients. As the disease is rare there is no consensus or RCT for therapy. There is data for use of clabirabine, 6MP and vincristine in multiple LN chain involvement. She should be seen by a hematologist for therapy options, preferably in a bigger academic center as this disorder is quite rare.

Already evaluated by one of the oldest Medical college,and biopsy was done there. She has only the report . No slides or pictures of biopsy. Already evaluated by Hematologist,Oncologist, pulmonologist,althougt she is asymptomatic.

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Rosai dorfman Common in neck region Consist of histeosite No danger to life Zavec

It's quite a rarity . Rosai Dorfman.

Tubercular lymphoma local infection

Thank you doctor
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