Random cases to test your knowledge!

1. Random cases to test your knowledge! Matt Frank M.D.

2. Let’s start with an easy one…

3. Classic Hodgkin lymphoma Nodular sclerosis Mixed cellularity Lymphocyte rich Lymphocyte depleted Nodular lymphocyte predominant Hodgkin lymphoma Hodgkin Lymphoma

4. Nodular Sclerosis Hodgkin Lymphoma • Collagen bands surrounding at least one nodule and lacunar type “HRS” cells • 70% of Classical Hodgkin Lymphoma (CHL) • Median age of 28 years • 80% Mediastinum • 10-40% EBV • M:F =1:1

5. Nodular sclerosis Hodgkin lymphoma, “Lacunar cells”

6. Mixed Cellularity Hodgkin Lymphoma • Mixed inflammatory background without nodular sclerosing fibrosis • 20-25% of CHL • Median age of 37 years • Usually peripheral nodes ( but 30% in spleen) • 70% male • HIV association • 75% EBV

7. Mixed Cellularity Hodgkin lymphoma

8. Lymphocyte rich Hodgkin lymphoma • Nodular (common) or diffuse lymphoid background with scattered HRS cells • 5% of all HL • Higher median age • 70% male • Usually peripheral nodes • Slightly better prognosis

9. Lymphocyte depleted Hodgkin lymphoma • Diffuse form of CHL rich in HRS cells and/or depleted in non neoplastic lymphs • Median age of 37 years • 75% male • Strongest HIV association • Abdomen, retroperitoneum, BM • EBV associated • Main differential (morphological) is ALCL or Anaplastic DLBCL

10. Nodular lymphocyte predominant Hodgkin lymphoma

13. Nodular Lymphocyte Predominant Hodgkin Lymphoma • Vaguely nodular architecture with small lymphs, histiocytes, and scattered “L&H” cells • Male, 30-50 years old • Cervical, axillary, or inguinal nodes • No EBV • Malignant cells CD 15 and 30 negative, CD45 positive (opposite of CHL) • Main differential is TCRLBCL

17. Malaria • Plasmodium vivax (benign tertian fever) • Plasmodium falciparum (malignant tertian fever) • Plasmodium ovale (benign tertian fever) • Plasmodium malariae (benign quartan fever)

18. P. vivax (rings) • Infects large pale red cells with large rings(> 1/3 size of RBC) • Single ring forms • Shϋffner’s dots • One nucleus

19. P. vivax (rings)

20. P. vivax with “amoeboid” trophozoites)

21. P. vivax (trophozoite)

22. P. vivax (schizont)

23. P. falciparum (rings) • Any size red cell but more often mature • Multiple small ring forms (less than 1/3-1/5 diameter of RBC) often with multiple nuclei • Tend to stick to RBC membrane (applique form) • Only ring form and gametocyte generally present

24. P. falciparum (rings)

25. P. falciparum (gametocyte)

26. P. ovale

27. P. malariae • Small red cells • Single rings and single nuclei • No Shϋffner’s dots • “Band like ameboid forms • Schizonts with <12 merozoites

31. Babesiosis • Northeast U.S. • Usually asymptomatic but can be life threatening in elderly and immunocompromised • Transmitted by Ixodes tick (what other disease does Ixodes transmit?) • Can have ring forms indistinguishable from Plasmodium species • Look for characteristic “maltese cross” rings

33. Anaplastic large cell lymphoma • T-cell lymphoma (Hodgkin is a B cell lymphoma) • 10-30 percent of childhood lymphomas • Males; First three decades of life • Can occur almost anywhere nodal and extranodal (but not skin or should be classified as cutaneous ALCL~much better prognosis) • ALK, EMA, and CD 30 positive • t(2;5), NPM/ALK fusion protein (not present in skin cases) • Several subtypes including “common”, lymphohistiocytic, and small cell

35. Alder-Reilly anomaly • Lot’s of azurophilic (purplish) granules filling cytoplasm of neutrophils but also seen in other cell lines • Distinguish from toxic change, Chediak-Higashi, etc. • Associated with mucopolysaccharidoses (Hunter’s, Hurler’s, others) • Can be isolated abnormality

37. “Sea blue” histiocytes in marrow • Totally non-specific • Thought to be secondary to increased bone marrow cell turnover • Can be feature of various inflammatory processes and inborn errors of metabolism (Niemann-Pick) • Can be associated with lymphoma, myeloproliferative, and myelodysplastic disorders • 30% of CML patients have them in marrow (think CML for board purposes)

38. “Sea blue” histiocyte or ???

40. Gaucher’s disease • Deficiency in glucocerebrosidase A • Glucocerebroside accumulates in macrophages throughout body with “crumpled tissue paper” inclusions but can be indistinguishable from “pseudo-Gaucher” cells in CML • Three types (I, II, and III) • Type I – most common, spares brain, late onset • Type II and III – much rarer, brain involved, younger onset, more severe than type I • Treatable disease

42. Acanthocytes • Non-specific • For board purposes, remember association with abetalipoproteinemia and McLeod phenotype (lack of Kx antigen) • Associated with Chronic Granulomatous Disease

46. Rosai-Dorfman disease • Usually painless cervical lymphadenopathy but ~30% are elsewhere • Unknown etiology (? HHV-7) • Most prevalent in teenagers and blacks but anyone can be affected • Often resolves spontaneously but can linger indefinitely • Chemotherapy for persistent cases can be offered

49. AML with inv (16) • AKA M4e • ~10% of AML • Monocytic and granulocytic differentiation with abnormal eosinophil component • Shows various myeloid and monocytic markers (CD 13, 33, 14, 11c, 64, MPO) • Better prognosis