Pancytopenia and Splenomegaly

Today we discussed a case of pancytopenia.

Click here for a previous blog and here for differentials of pancytopenia based on marrow cellularity.

In general, it is still a useful approach to consider causes based on the premise of decreased production, which include primary marrow disorders such as malignancy, fibrosis, storage disease, as well as myelodysplasia and aplastic anemia (congenital or acquired including viral illness parvo b19, ebv). Nutritional deficiencies such as B12 and Folate, as well as sepsis are other reasons for decreased production leading to pancytopenia.

Our patient was feeling generally unwell with persistent nausea and vomiting and constitutional symptoms. 

Physical exam was significant for splenomegaly.

CT imaging showed mediastinal adenopathy, and confirmed the presence of an enlarged spleen.

Sidenote:

The causes of massive splenomegaly (spleen >1000 g):

• infectious: visceral leishmaniasis (kala-azar), malaria
• myeloproliferative: CML, myelofibrosis
• malignancy: primary lymphoma of spleen

Bone marrow aspiration was unsuccessful; however peripheral blood film showed atypical lymphocytes, and flow was consistent with a B cell lymphoma. 

The patient will be followed up as an outpatient to facilitate formal tissue diagnosis, staging and subsequent management. 

Quick word on hematologic malignancies:

Lymphomas are classically subdivided based on Hodgkin's and Non-hodgkin's lymphoma. 

Within NHL, most helpful to think of in terms of indolent (e.g. follicular, marginal zone, mantle cell) and aggressive (e.g. DLBCL), and very aggressive (e.g. Burkitt's lymphoma)

As a general principle, indolent lymphomas are not curable, but slow growing, and aggressive lymphomas are curable, but as the name implies, more aggressive.

Treatment will likely consist of chemotherapy and steroids. Because the patient will be immunosuppressed, the last point of the day was to remember to consider latent infections and so to screen for:
- TB
- Hepatitis B
- HIV
(- Strongyloides which can in the face of immunosuppresion can lead to disseminated hyperinfection)